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4384 

SYPHILIS  AND  THE 
NERVOUS  SYSTEM 


FOR 


PRACTITIONERS,  NEUROLOGISTS 
AND  SYPHILOLOGISTS 


BY 

DR.  MAX  NONNE 

CHIEF   OF   THE    NERVOUS    DEPARTMENT    IN    THK    GENERAL    HOSPITAL,    HAMBURG.    EPPENDORF 


AUTHORIZED  TRANSLATION  FROM  THE  SECOND 
REVISED  AND   ENLARGED  GERMAN   EDITION 

BY 

CHARLES  R.  BALL,  B.A.,  M.D. 

CHIEF  OF  THE  NERVOUS  AND  MENTAL  DEPARTMENT,  ST.  PAUL  FREE  DISPENSARY;  NEUROLOGIST, 

ST.  JOSEPH  HOSPITAL.  BETHESDA  HOSPITAL,  MOUNDS  PARK  HOSPITAL,  MINNESOTA 

SOLDIERS'  HOME,  AND  STATE  HOME  FOR  CRIPPLED  AND  DEFORMED  CHILDREN. 


98  ILLUSTRATIONS  IN  TEXT 


PHILADELPHIA  &  LONDON 
J.  B.  LIPPINCOTT  COMPANY 


COPYRIGHT,    1913 
BY  J.    B.   LIPPINCOTT  COMPANY 


EwaedicaJ 

library 

WC 


DEDICATED 
To  His  TEACHER 

PROFESSOR  WILLIAM  ERB 

WITH    REVERENCE    AND    GRATITUDE 
BY 

THE  AUTHOR 


624006 


PREFACE  TO  THE  SECOND  EDITION 


IN  the  six  years  since  the  appearance  of  the  first  edition 
of  "Syphilis  and  the  Nervous  System,"  important  new 
discoveries  have  been  added  to  this  subject.  The  discovery 
of  the  Spirochtete  pallida  by  Schaudinn,  whose  premature 
death  has  been  a  great  loss  to  scientific  investigation,  the 
beginning  and  development  of  cytodiagnosis,  as  well  as 
the  globulin  examination  of  the  spinal  fluid,  and  finally 
the  application  of  the  complement-deviation  method  for  the 
diagnosis  of  syphilis  by  Wassermann  and  A.  Neisser  and 
the  utilization  of  these  methods  by  neurologists,  must  deeply 
affect  the  theme  ' '  Syphilis  and  the  Nervous  System. ' '  The 
discussion  concerning  lues  nervosa  in  the  first  edition  was 
only  briefly  touched  upon  in  a  purely  clinical  manner.  It 
now  demands  a  thorough  consideration,  also  the  gradually 
increasing  number  of  cases  of  recovery  or  of  a  quiescence 
of  the  symptom  complex  of  paresis  warns  us  that  we, 
mutatis  mutandis,  must  reconstruct  our  symptomatology 
and  prognosis  of  that  disease  as  we  have  already  done  for 
tabes.  I  have  endeavored  in  the  second  edition  to  set  forth 
these  results  of  investigation  and  practice. 

Since  the  entire  arrangement  of  the  material  has  proven 
to  be  a  practical  one,  I  have  found  no  occasion  to  change 
the  outline  in  its  entirety. 

The  last  six  years  have  brought  to  me  an  abundant 
material  of  syphilogenetic  diseases.  I  have  used  this  mate- 
rial for  illustration  whenever  it  has  served  to  bring  out 
new  view-points  and  in  the  discussion  of  old  and  new 
questions  of  contention  helped  to  shed  new  light.  Notwith- 
standing, the  book  maintains  the  character  which  I  have 
desired  to  impart  to  it  from  the  beginning,  namely,  the 
stamp  of  the  personal,  individual  experiences  of  a  clinician. 
The  pertinent,  in  recent  years  extremely  voluminous,  litera- 
ture I  have  reviewed  as  far  as  possible.  I  believe  this  time 


vi  PREFACE 

that  I  have  not  omitted  the  consideration  of  the  essential 
and  important  contributions  to  this  subject. 

I  wish  to  thank  my  assistants  for  the  support  which 
they  have  given  me.  I  desire  to  thank  especially  Doctors 
Apelt,  Eichelberg,  and  Suntheim,  without  whose  efficient 
and  conscientious  assistance  I  should  not  have  been  able 
to  present  the  material  for  this  book. 

The  book  may  be  said  to  have  been  written  out  of  the 
practice  for  the  practice.  For  this  reason  I  regard  the 
citation  of  individual  cases  illustrating  the  form  of  disease 
described  as  indispensable.  I  have  endeavored  to  present 
the  case  histories  in  as  brief  and  concise  a  manner  as 
possible  with  the  hope  that  they  will  be  carefully  read. 
I  hope  also  that  the  practitioner  seeking  for  advice  will 
find  among  them  similar  cases  to  his  own.  May  the  kindly 
reception  which  was  given  to  the  first  edition  by  my  profes- 
sional colleagues  also  be  accorded  to  the  second  edition. 

DR.  NONNE. 


TRANSLATOR'S  PREFACE 


WHILE  a  student  of  Dr.  Nonne's  in  Hamburg,  I  con- 
ceived the  idea  of  translating  his  book  "Syphilis  and  the 
Nervous  System"  into  English.  The  book  as  an  authority 
on  this  subject  stands  very  high  in  Germany  and  has  been 
translated  into  Italian.  I  have  endeavored  to  present  the 
subject  matter  of  the  original  in  a  somewhat  briefer  form 
in  the  translation.  In  order  to  do  this  I  have  omitted  in 
the  translation  chiefly  two  things :  first,  extensive  references 
to  different  authorities,  and,  second,  many  case  reports. 
The  subject  matter  itself  has  been  shortened  very  little. 

Since  the  subject  of  the  book  "Syphilis  and  the  Nervous 
System"  is  in  such  a  state  of  transition  at  the  present  time, 
due  chiefly  to  the  four  reactions  and  salvarsan  therapy, 
Dr.  Nonne  has  rewritten  for  this  edition  of  his  book  the 
last  two  chapters  of  the  original  book  on  the  four  reactions, 
and  has  added  a  chapter  on  salvarsan  therapy.  This  brings 
the  whole  subject  up  to  date. 

CHARLES  B.  BALL. 
ST.  PAUL,  MINN. 


Vll 


CONTENTS 


PAGE 

I.  INTRODUCTION    1 

Importance  of  a  Knowledge  of  Syphilis  of  the  Nervous  System  1 

Increase  of  Syphilis 1 

Increase  of  Nervous  Disease  in  General 2 

Frequency  of  Syphilitic  Disease  of  the  Nervous  System 3 

Historical   4 

Gummatous  Processes  and  Heubner's  Arteritis 4 

Symptoms  in  Themselves  Not  Characteristic ' 5 

The  Infectious  Factor   5 

The  Transmission  of  Syphilis  to  Monkeys 6 

Schaudinn's    Spirodi*te  Pallida 6 

Proof  of  Specific  Infection  and  Its  Difficulties 7 

Ignorance  of  the  Infection 8 

Extragenital   Infection    8 

Syphilitic  Findings  Postmortem  with  Negative  History 9 

Examination  for  Past  Evidences  of  Syphilis 10 

Not  Every  Organic  Nervous  Disease  in  a  Person  Infected  with 

Syphilis  is  of  Specific  Origin  11 

There  May  Also  be  a  Combination  of  Several  Etiological  Fac- 
tors    11 

Diagnostic  Importance  of  Antispecific  Therapy 12 

Summary 13 

II.  PATHOLOGY 15 

Cranium  and  Vertebrae   15 

Syphilis  of  the  Vertebrae 16 

Pathology  of  Nervous  Syphilis   17 

Gummata    18 

Regressive  Metamorphosis    26 

Secondary  Changes  in  the  Brain  Substance   26 

Syphilitic  Meningitis,  Gummatous  and  Fibrous  Hyperplastic 

Form    28 

Pia  Mater 28 

Simple  Meningitis 30 

Localization  of  the  Meningitis 30 

Injury  to  the  Structures  on  the  Base  . 31 

Disease  of  the  Blood- Vessels  and  Arteries 33 

Arteriosclerosis  in  Syphilitics   38 

The  Syphilitic  Aortitis  of  Heller-Dohle 40 

Aneurism    41 

Summary  of  the  Vessel  Disease  in  Syphilis 41 

Eesults  of  Disease  of  the  Blood-Vessels  in  Cerebral  Lues 42 

Rupture  and  Secondary  Hemorrhages   43 

Combination  of  Different  Pathological  Forms  44 

ix 


CONTENTS 

Differential  Diagnosis  in  Specific  Processes   44 

Sarcoma    44 

Cysticercus  44 

Tuberculosis 45 

Primary  Disease  of  the  Nervous  System  47 

III.  ETIOLOGY  OF  NERVOUS  LUES  AND  SPECIFIC  ENDABTERITIS 50 

Frequency  of  Nervous  Involvement  in  the  Early  Stages 50 

Late  Development 50 

Syphilis  Acquired  Late  in  Life 51 

Syphilitic  Virus  with  Special  Toxicity  for  the  Nervous  System  52 

Objections  to  the  Theory  of  a  Special  Toxicity   54 

Ehrmann's  Theory   54 

Influence  of  Treatment   56 

Influence  of  the  Severity  of  the  Course  on  Nervous  Involve- 
ment    58 

Influence  of  Extragenital  Infection 59 

Influence  of  Head  Injuries   59 

Influence  of  Mental  Work 59 

Influence  of  Alcohol  59 

Neuropathic  Heredity   60 

Symptomatology  of  the  Arteritic  Form  of  Cerebral  Syphilis. .  .  60 

The  Time  of  Occurrence 60 

Age  of  the  Patients 61 

Prodromal  Symptoms 61 

Headache    62 

Dizziness 62 

Insomnia     62 

Psychic  Disturbances   62 

Intoxicated-like  State  of  Heubner 63 

Arterial  Disease  on  the  Base  of  the  Brain 65 

Variations   in  Blood-pressure    66 

Symptoms  of  Motor  Irritation 66 

Transient  Paralysis   66 

Apoplectic  Symptoms 67 

Triplegia 68 

Pons  Involvement 68 

Sensation    69 

Hemianopsia 69 

Course   70 

Diagnosis    72 

Differential  Diagnosis  Apoplexy   72 

Brain  Tumor   73 

Multiple   Sclerosis    73 

Bright's  Disease    73 

Hysteria    73 

General  Paresis 74 

Tuberculosis 74 

Heart  Disease  in  Syphilitics 74 

Arteriosclerosis  in  Young  Syphilitics 74 

Summary    75 


CONTENTS  xi 

IV.  SYPHILITIC  CEREBRAL  MENINGITIS   76 

Incompleteness  and  Transient  Character  of  the  Clinical  Symp- 
toms     76 

Multiplicity   of    Symptoms    77 

Symptoms  of  Specific  Meningitis  of  the  Convexity 78 

Headache    78 

Psychic  Anomalies   79 

Case   of   Slow-developing  Progressive  Dementia,   without   So- 
matic Symptoms,  Cured  by  Antispecific  Therapy 80 

Pupil  Anomalies   81 

Combination  of  Paresis  with  Specific  Meningitis  of  the  Con- 
vexity      83 

Choked  Discs 84 

Cortical  Convulsions    84 

Hemiepilepsy     85 

Case  of  Hemiparesis  Apoplectica  Arteritica 87 

Meningitis  Convexitatis  Luetica  Post-traumatica 88 

General  Epileptic  Convulsions  90 

Cortical  Disturbances  of  a  Cortical  Origin 90 

Cortical   Speech  Disturbances    90 

Hemianopsia    91 

Differential    Diagnosis    of   Meningitis    Syphilitica    Cerebralis 

Corticalis.     Headache  from  Local  Causes   91 

Simple  Headache    91 

Headache  due  to  Uraemia  91 

Headache  with  Pachymeningitis  Alcohol ica  92 

Tumor  Cerebri 92 

Brain  Abscess   93 

Specific  Arteritis    93 

Paresis    94 

Cerebral   Arteriosclerosis    94 

Tuberculosis   94 

V.  SPECIFIC  BASILAR  MENINGITIS 96 

Clinical  Symptoms   96 

Psychic  Disturbances   96 

Temperature 97 

Polyuria  and  Polydipsia   97 

Cranial  Nerves    97 

Involvement  of  the  Olfactory  Nerve   97 

Optic  Nerve  97 

Ophthalmoscopic  Changes  in  Choked  Disc  99 

Explanation  of  Choked  Discs   100 

Optic  Neuritis  100 

Neuritis  Descendens  100 

Combination  of  Different  Ophthalmoscopic  Changes   100 

Disturbances   of   Vision    101 

Form  of  Visual  Disturbance 101 

'   In  Marked  Disease  of  the  Optic  Nerve  Some  Vision  is  Usually 

Retained    102 

Irregularities  in  Field  of  Vision 102 


xii  CONTENTS 

Atrophy  of  the  Optic  Nerve  with  Double  Neuritis 102 

Ophthalmoscopic  and  Perimetrical  Examination  Necessary.  .  103 

Spinal  Form  of  Optic  Atrophy  in  Tabes 103 

Influence  of  Treatment  105 

Pseudo-brain  Tumors  106 

Changes  in  the  Visual  Power  107 

Oscillation  of  Sight  in  a  Sarcoma  of  the  Pia  Sheath  of  the 

Optic  Nerve  107 

Uhthoff's  Experience  Concerning  Disease  of  the  Optic  Nerve 

in  Brain  Syphilis  108 

Choked  Disc 109 

Optic  Neuritis  110 

Optic  Atrophy  110 

Visual  Disturbances  110 

Chiasm  and  Optic  Tract  110 

Oscillating  Hemianopsia  112 

Basal  Bitemporal  Hemianopsia,  Descending  Neuritic  Atrophy 

on  One  Side 112 

Basal  Homonymous  Left-sided  Hemianopsia,  Together  with 

Abducens  Paralysis  on  the  Right  Side 113 

Hemioptic  Reaction  of  the  Pupils  113 

Hemianopsia,  Hemianoptic  Pupil  Reaction,  Basal  Paralysis 

of  the  Eye  Musculature,  and  Epilepsy   114 

VI.  SYMPTOMATOLOGY  OF  SYPHILIS  OF  THE  BASE  OF  THE  BRAIN 116 

Frequency  of  Paralysis  of  the  Eye  Muscles  in  Lues  of  the 

Nervous   System    116 

Course  of  the  Oculomotor  Nerve    116 

Subdivisions  of  the  Oculomotor  Nucleus 117 

Blood  Supply  of  the  Oculomotor 118 

Separate  Course  of  the  Root-fibres   119 

Course  of  the  Trochlear  Nerve  119 

Course  of  the  Abducens 119 

Is  Paralysis  of  the  Eye  Muscle  Pathognomonic  of  Lues? 119 

Meningitis  Basalis  Luetica    122 

General  Description  of  the  Paralysis  of  the  Eye  Musculature 

in  Nervous  Syphilis  126 

Early  Appearance  of  Paralysis   127 

Late  Appearance  of  Paralysis   127 

Frequency  of  Oculomotor  Paralysis    127 

Complete  Paralysis  of  the  Oculomotor  Nerve   128 

Mauther's  Theory  of  Nuclear  and  Peripheral  Paralysis 128 

Perineuritis  of  Individual  Intraorbital  Branches  of  the  Oculo- 
motor    128 

Secondary  Disease  of  the  Oculomotor  in  the  Orbital  Fissure. .  128 

Disease  of  the  Oculomotor  in  the  Nucleus  and  Root  Area 129 

In  Disease  of  the  Oculomotor  Trunk  Isolated  Paralysis  May 

Occur    129 

Involvement  of  the  Internal  Oculomotor  Branches 129 

Pupil  Anomalies  in  Normal  Persons .  130 


CONTENTS  xiii 

Pupil  Anomalies  in  Syphilitics   130 

Anomalies   in   the   Reaction   of   the   Pupils   in   Persons   Who 

Have  Never  Had  Syphilis  Have  Frequently  Been  Observed.  131 

Ophthalmoplegia  Interna    133 

A  Precursor  of  Postsyphilitic  Disease 133 

Summary  in  Oculomotor  Paralysis  Occurring  in  Syphilis....  135 

Disease  of  the  Abducens 135 

Paralysis  of  the  Trochlear   . .  .  ." 136 

Nystagmus  136 

VII.  SYMPTOMATOLOGY  AND  PROGNOSIS  OP  SPECIFIC  BASILAB  MENIN- 
GITIS    137 

The  Differential  Diagnosis  of  the  Paralysis  of  the  Eye  Muscles 

in  Cerebrospinal  Lues  and  Tabes  Dorsalis 137 

Tabes  Dorsalis 137 

Progressive  Ophthalmoplegia  138 

Brain  Tumor  on  the  Base 138 

Meningitis  Cysticercus    140 

Multiple  Sclerosis 140 

Cerebral  Hemorrhages   141 

Head  Injuries    141 

Tubercular  and  Epidemic  Meningitis  141 

Relapsing  Oculomotor  Paralysis   141 

Trigeminal  Nerve   142 

Involvement  of  the  Facial  Nerve 143 

Disease  of  the  Eighth  Nerve   145 

Meniere's  Symptom-complex 147 

Glossopharyngeal  Nerve    147 

Symptoms  Caused  by  Disease  of  the  Vagus   148 

Hypoglossal  Nerve   148 

Pons  and  Medulla 149 

Blood   Supply    149 

Medulla  150 

Glycosuria  and  Polyuria 151 

Diabetes  Mellitus   151 

Diabetes  Insipidus   153 

Prognosis  of  Brain  Syphilis 153 

Statistics 154 

A  Partial  Recovery   155 

Recurrences    155 

Age  of  Patient  at  Time  of  Infection   155 

Interval  between  the  Time  of  the  Infection  and  the  Outbreak 

of  the  Brain  Affection 156 

Heredity,  a  Bad  Nervous  Inheritance 156 

Debilitating  Agents   156 

Head  Injuries    156 

Form  and  Degree  of  the  Primary  and  Secondary  Symptoms.  .  157 

Extragenital  Infection 157 

Previous  Treatment   158 

Difference  between  Clinical  and  Anatomical  Recovery   159 

Prognosis  in  Localized  Meningitis   159 


xiv  CONTENTS 

Diffuse  Meningeal   Infiltration    159 

Isolated  Gummata    159 

Arteritis 160 

Summary 161 

VIII.  NEUROSES    AND    PSYCHOSES    IN    SYPHILITICS    AND    IN    CEREBRAL 

SYPHILIS    166 

Historical   166 

Different  Ways  in  Which   Syphilis  Causes  Psychic  Disturb- 
ances      168 

Changes  in  the  Blood 168 

Dyscrasia  as  a  Result  of  Antispecific  Treatment 169 

Disease  of  the  Blood  Channels   169 

Influence  of  the  Infection  Itself  upon  the  Mental  Condition. .  169 

Native  Resistance  of  the  Person  Infected 170 

Functional  Nervousness    170 

Cerebral  Neurasthenia 171 

Hysteria    171 

Chorea 172 

Epilepsy 172 

Hypochondria 174 

Melancholia    175 

Mania 175 

Manic-depressive  Insanity    176 

Paranoia 178 

Catatonia 178 

Amentia 1 79 

Dementia 179 

Korsakoff's  Symptom-complex   180 

There  is  No  Mental  Disturbance  Characteristic  of  Syphilis.  .  .  180 

IX.  DEMENTIA  PARALYTICA  AND  SYPHILIS   182 

Increase  of  Paresis 182 

Earlier  Development  of  Paresis 182 

Increase  among  Women    182 

Other  Causes  of  Paresis   183 

Syphilis 183 

Statistics 183 

Infantile  and  Juvenile  Paresis   185 

Late  Syphilis — Late  Paresis    185 

Combination  of  Paresis  and  Syphilitic  Brain  Disease 186 

Krafft-Ebing's  Experiments   186 

Opponents  of  This  Doctrine 187 

Neuropathic    Inheritance    187 

Pathology   188 

Antispecific  Treatment    190 

Consensus  of  Opinion  Concerning  the  Relationship  of  Paresis 

and   Syphilis    190 

Spielmeyer's    Work    190 

Differential  Diagnosis   191 

Diffuse  Syphilitic  Meningitis    191 


CONTENTS  xv 

Arteritic  Form  of  Brain  Syphilis    192 

Is  Recovery  in  Paresis  Possible? 193 

Syphilitic  Pseudoparesis   196 

Combination  of  Paresis  and  Brain  Syphilis   198 

Arteriosclerotic  Brain  Disease  in  Former  Syphilitics   198 

Diffuse  Arteriosclerotic  Brain  Disease  in  a  Syphilitic 198 

Encephalomalacia  in  Syphilitics   201 

Cerebral  Neurasthenia 202 

Chronic  Alcoholism    203 

Brain-tumors    204 

Syphilis  and  Migraine    204 

X.  SYPHILIS  OF  THE  SPINAL  CORD  207 

Rareness  of  Lues  When  Confined  to  the  Spinal  Cord  Alone.  .  207 

Spinal  Syphilis  May  Appear  Early 208 

Late  Appearance  of  Spinal  Lues   208 

The  Previous  Syphilis  May  Have  Been  Slight  or  Severe 208 

Men  More  Frequently  Affected  than  Women   208 

Influence  of  Therapy 209 

Influence  of  Age   209 

The  Peculiarities  of  Spinal  Lues  Are  Due  to  the  Anatomical 

Peculiarities  of  the  Cord 209 

The  Pathology 210 

Syphilis  of  the  Vertebrae   210 

Syphilis  of  the  Membranes 211 

Disease  of  the  Blood-vessels  214 

The  Cord  Itself  is  Not  Primarily  Affected  214 

Chronic  Myelitis 215 

Gummata  in  the  Substance  of  the  Cord   215 

Disease  of  the  Gray  Substance  215 

Transverse  Myelitis   216 

Root  Neuritis    216 

System  Diseases    218 

The  Pathology  of  Spinal  Syphilis  in  Itself  Not  Characteristic.  219 

Symptomatology,  Multiplicity  of  Symptoms   220 

The  Meningeal  Symptom-complex  220 

Meningeal  Irritation    221 

Spinal  Meningitis   221 

XI.  MENINGOMYELITIS  224 

Meningomyelitis  the  Most  Frequent  Form  of  Spinal  Syphilis.  .  224 

Arterial  Distribution 224 

The   Clinical   Symptoms   Depend   upon   the   Location    of   the 

Arterial  Disease  224 

Lumbar   Myelitis    227 

Cervical  Myelitis    227 

Specific  Cervical  Pachymeningitis,  Chronic  Dorsal  Myelitis.  .  229 

Course  of  Meningomyelitis   231 

Erb's  Syphilitic  Spinal  Paralysis    232 

Erb's  Definition    .                                                              232 


xvi  CONTENTS 

"*•  XII.  SYPHILIS  OF  THE  SPINAL  CORD  236 

Acute  Specific  Transverse  Myelitis 236 

Frequency 236 

Mode  of  Appearance 236 

Sensation    236 

Prodromal   Symptoms    237 

The  Course 237 

Differential  Diagnosis,  Tuberculosis 240 

Malignant  Tumor   241 

Spinal  Apoplexy   241 

Pathology   241 

Incongruence  between  the  Pathology  and  the  Clinical  Symp- 
toms     242 

Simple  Acute  Myelitis  in  Syphilitics 243 

Landry's  Paralysis  244 

Disease  of  the  Anterior  Gray  Substance 245 

Amyotrophic  Lateral  Sclerosis   248 

Syringomyelia    248 

Central  Gliosis  with  Disease  of  the  Posterior  Columns 249 

The  Brown-Sequard  Symptom-complex   249 

Symptoms  of  Multiple  Sclerosis 252 

Symptoms  of  Tumor   255 

Symptoms  of  Combined  Tract  Disease 255 

Spinal  Koots   257 

XIII.  TABES  AND  SYPHILIS    259 

The  Relationship  of  Tabes  and  Syphilis   259 

Historical 259 

Arguments  for  the  Relationship   259 

Variation  in  Statistics 260 

Conjugal  Tabes   261 

The  Later  Development  of  Tabes   263 

The  Frequency  of  Sterility  in  Tabetics   263 

Tabes  in  Hereditary  Lues    264 

Tabes  and  Syphilitic  Diseases  in  the  Same  Family 264 

Combination  of  Tabes  with  Syphilitic  Disease  of  the  Nervous 

System  and  Internal  Organs   265 

A  Combination  of  Specific  Spinal  Meningitis  and  Tabes 266 

Striimpell's  Toxin  Theory   266 

The   Position   of   the   Opponents    in   the   Tabes-syphilis    Dis- 
cussion    267 

Marie's  Theory    268 

The  Antispecific  Therapy  as  an  Argument  is  Useless 269 

The  Infrequency  of  Tabes  in  Prostitutes 269 

The  Disproportion  between  the  Frequency  of  Syphilis  and  the 

Infrequency  of  Tabes  in  Different  Countries 270 

Edinger's  Exhaustion  Theory    271 

Atypical  Types  of  Tabes 273 

Lightning  Pains  as  an  Isolated  Symptom 273 

Absence  of  the  Patellar  Reflexes 273 

Isolated  Pupil  Anomalies   274 


CONTENTS  xvii 

Isolated  Gastric  Crises   274 

Pseudotabes   Syphilitica 275 

Differential  Diagnosis  of  Spinal  Syphilis.     History 277 

Proof  in  the  History  of  an  Infection  277 

Other  Symptoms  of  Syphilis   277 

The  Intensity  of  Individual  Symptoms  No  Criterion  to  Judge 

of  Local  Involvement 277 

The  Brown-Sequard  Symptom-complex   277 

Brain  Involvement   . 277 

Irritative  Symptoms 277 

Transient  Character  of  Symptoms   278 

Influence  of  Therapy   278 

Spinal  Neurasthenia    278 

Compression  Myelitis  from  Tubercular  Caries 278 

Spinal-cord  Tumors    279 

Multiple   Sclerosis 280 

Combined  Tract  Disease   281 

Hysteria    282 

Prognosis  of  Spinal  Syphilis 282 

General 282 

Prognosis  Depends  upon  the  Form  of  Spinal  Disease 283 

Prognosis  Not  Influenced  by  Previous  Treatment 284 

Relapses    284 

>  XIV.  CEREBROSPINAL  FORM  OF  SYPHILIS 285 

Either  Brain  or  Spinal  Symptoms  Frequently  Predominate. . .  285 

Is  Involvement  of  the  Brain  More  Often  Latent  ? 285 

Pathology   286 

The  Postsyphilitic  Changes 286 

Recapitulation  of  Symptoms   294 

Differential  Diagnosis   295 

Multiple   Sclerosis    295 

Differential  Diagnosis  in  Tuberculosis 296 

Sarcoma  and  Carcinoma  of  the  Leptomeninges 296 

Cysticerci    297 

•£  XV.  SYPHILITIC  DISEASE  OF  THE  PERIPHERAL  NERVES   298 

Secondary  Disease  of  the  Peripheral  Nerves  Caused  by  Dis- 
ease of  the  Bones,  Lymph-glands,  Fascia,  and  Muscles 298 

Primary  Disease  of  the  Peripheral  Nerves  299 

Syphilitic  Root  Neuritis   299 

Peripheral  Perineuritis   300 

Simple  Specific  Degenerative  Polyneuritis   301 

Neuralgias    301 

Neuralgia  of  the  Trifacial   301 

Neuralgia  of  the  Cervical  Plexus  302 

The  Brachial  Plexus 302 

Intercostal   Neuralgia    302 

Neuralgia  in  the  Region  of  the  Lumbar  and  Sacral  Plexus ....  302 

Syphilitic  Neuritis  and  Polyneuritis   303 


xviii  CONTENTS 

Polyneuritis   304 

Polyneuritis  in  a  Patient  with  Probable  Congenital  Syphilis.  .  306 
Multiple  Specific  Root  Neuritis  307 

j£XVI.  HEREDITARY  SYPHILIS  AND  THE  NERVOUS  SYSTEM 310 

Extra-uterine  Infection  310 

The  Spirochsete  Pallida  310 

Fundamentals  in  Hereditary  Lues  311 

The  Attenuation  of  the  Specific  Virus  311 

Severity  of  Parental  Syphilis  Does  Not  Always  Correspond 

to  the  Degree  of  Congenital  Lues  312 

Syphilis  of  Parents  May  Have  Occurred  a  Long  Time  Back.  .  312 
Frequency  of  Involvement  of  the  Various  Internal  Organs  in 

Hereditary  Syphilis  312 

Late  Hereditary  Syphilis  313 

Time  of  Appearance  of  the  Late  Symptoms 313 

Symptoms  of  Hereditary  Syphilis  Apart  from  the  Nervous 

System 313 

Eye  Symptoms  314 

Pathology  of  the  Nervous  System  in  Hereditary  Syphilis.  .  .  .  314 

Defective  Development  315 

Hydrocephalus  315 

Brain  Apoplexy  317 

Disease  of  the  Blood-vessels 317 

Disease  of  the  Meninges  317 

True  Specific  Nervous  Disease  in  Congenital  Syphilis  in 

Combination 317 

Isolated  Spinal-cord  Syphilis  Probably  Does  Not  Occur  in 

Congenital  Lues 318 

Summary  of  the  Pathological  Changes  318 

Clinical  Symptoms  319 

General  Debility  or  Lack  of  Vitality 319 

Nervous  Form  of  Rickets 319 

Simple  Nervousness  320 

Hysterical  and  Hysteroid  Conditions  321 

Migraine  May  Also  be  an  Expression  of  Congenital  Syphilis.  .  322 

Psychoses  322 

Symptoms  of  Hydrocephalus  322 

Symptoms  of  Arteritis 322 

Mental  Weakness  and  Idiocy 322 

Epilepsy  323 

Basilar  Cranial  Nerve  Paralyses  324 

Paralysis  of  the  Eye-muscles  Less  Frequent  in  the  Congenital 

Than  in  Acquired  Lues  324 

Isolated  Loss  of  the  Light  Reaction  324 

Polyuria  and  Glycosuria  325 

The  Optic  Nerve 325 

Deafness  326 

Complicated  Cerebrospinal  Symptoms  326 

Hereditary  Syphilitic  Pseudoparalyses  326 


CONTENTS  xix 

Differential  Diagnosis 328 

Alcoholism  of  the  Parents , 328 

Tuberculosis  of  the  Central  Nervous  System 329 

Metasyphilitic  Diseases  of  the  Nervous  System  in  Congenital 

Lues.     Tabes  Dorsalis     329 

Pseudotabes   Syphilitica 331 

Dementia  Paralytica    331 

Homen's  Familiar  Disease   (Idiocy  with  Spastic  Paralysis)  .  .   332 
Spastic   Spinal  Paralysis  with   and   without  Cerebral   Symp- 
toms     333 

Sachs's  Amaurotic  Idiocy    334 

Disseminated   Sclerosis    335 

Symptoms  of  Friedreich's  Ataxia   335 

Congenital  Syphilis  in  the  Third  Generation   336 

XVII.  CONCERNING  THE  BEHAVIOR  OF  THE  WASSERMANN  REACTION  IN  THE 
BLOOD  AND  SPINAL  FLUID,  ALSO  PLEOCYTOSIS  AND  THE  INCREASE 
OF  GLOBULIN  (PHASE  I)  IN  SYPHILOGENETIC  DISEASES  OF  THE 

NERVOUS  SYSTEM 338 

Introduction  338 

The  Wassermann  Reaction  Not  a  Specific    338 

Examination  of  Cerebrospinal  Fluid   338 

The  Technique  of  Lumbar  Puncture 339 

Dangers  and  Unpleasant  After-effects  of  Lumbar  Puncture. .   339 

The  Presence  of  Blood  in  the  Cerebrospinal  Fluid 340 

The  Globulin  Test.     Phase  I   341 

The  Fuchs-Rosenthal  Method  of  Counting  the  Lymphocytes..   341 
Technique  of  Obtaining  Blood  for  the  Wassermann  Reaction.  .   342 

The  Wassermann  Reaction    342 

Principle  of  the  Reaction   343 

Technique  of  the  Reaction   344 

The  Significance  of  Lymphocytosis 345 

The  Origin  of  the  Cellular  Elements  346 

The  Significance  of  Phase  I    346 

The  Wassermann  Reaction  in  Recent  Cases  of  Syphilis  with- 
out Nervous  Symptoms    347 

The  Significance  of  the  Wassermann  Reaction  in  the  Blood.  . .   348 

General  Paralysis  349 

The  Wassermann  Reaction  in  the  Spinal  Fluid 349 

The  Value  of  the  Four  Reactions  in  Differential  Diagnosis. .  .   351 

Typical   Findings    352 

The  Importance  of   the  Four   Reactions   in  Determining  the 

Recovery  of  the  Patient 353 

The  Significance  of  Haemolysin 354 

The  Four  Reactions  Do  Not  Solve  All  the  Questions  of  Diag- 
nosis    354 

The    Absence    of    the    Wassermann    Reaction    in    Syphilitic 

Arteritis    354 

The  Importance  of  a  Thorough  Clinical  Examination   354 


xx  CONTENTS 

XVIII.  PROPHYLAXIS    356 

Physical  and  Psychic  Trauma,  Alcoholism   356 

Intelligent  Treatment  of  the  Primary  and  Secondary  Lesions.  357 

Treatment  with  Mercury  and  lodid   358 

Inunctions,  Injections,  Sack-therapy,  and  Internal  Medication  358 

Lewin's    Sublimate   Injection    358 

Mercurial  Neuritis   361 

Treatment  by  Inunctions    361 

Indications  for  Mercury  and  lodid 362 

Action  of  Mercury  in  Non-specific  Affections  363 

Duration  of   Specific  Treatment  Where  the   Symptoms   Con- 
tinue Refractory  364 

Chronic  Intermittent  Treatment  365 

Antimercurialism    366 

Refractory  Behavior  of  Genuine  Specific  Processes  to  Mercury 

and  Iodine   366 

Optic  Atrophy  a  Contraindication   366 

Atoxyl 367 

Bath  Resorts  367 

Zittman   Cure    367 

Tonic  Measures 368 

Psychic  Treatment   368 

Therapeutic    Indications    for    Individual    Forms    of    Nervous 

Lues 368 

Surgical  Treatment 370 

XIX.  SALVARSAN  THERAPY 376 

Introduction   376 

Dangers   376 

Intramuscular  and  Subcutaneous  Injections   376 

The  Intravenous  Method 377 

The  Influences  of  Salvarsan  on  Syphilogenetic  Diseases 382 

Recapitulation   383 

Tabes  Dorsalis 384 

Paresis    384 

Dosage  384 

Salvarsan  in  Congenital  Syphilis   385 

BIBLIOGRAPHY  387 


ILLUSTRATIONS 


FIG.  PAGE 

1.  Cut  from  the  left  paracentral  lobe.  Under  the  raised  dura  is  a  gumma- 

tous  tumor  (Nonne) 18 

2a.  A  hard  fibrous  gumma  on  the  base  of  the  right  frontal  lobe,  growing 

out  from  the  dura  (Nonne) 19 

26.  Gummata  on  the  inner  surface  of  the  dura  in  the  right  cranial  convexity  20 

3a-36.  Gumma  of  the  dura  (Nonne) 21 

4.  Multiple  gummata  on  the  base  of  the  brain  (Baumgarten) 22 

5.  Gummata  of  the  optic  tract  and  pons  (Cornil) 23 

6.  Syphilis  of  the  central  nervous  system  (Lang) 23 

7.  Gummatous  changes  on  the  base  of  the  brain  (Siemerling) 23 

8.  Encephalitis  gummosa  (Straiissler) 24 

9.  Encephalitis  gummosa.    Miliary  gummata  with  giant  cells  (Straiissler) .  24 
10-11.  Encephalitis  gummosa  (Nonne) 25-26 

12.  Vessels  in  a  brain  gumma,   surrounded  by  small-celled  infiltration 

(Nonne) 27 

13.  Leptomeningitis  cerebralis  luetica  (Nonne) 29 

14.  Leptomeningitis  chronica  with  general   cloudiness  of  the  soft  men- 

inges  (Bechterew) 31 

15a.  Leptomeningitis  basalis  chronica  (Nonne) 32 

156.  Leptomeningitis  basalis  syphilitica.  Transverse  section  from  the  region 

of  the  corpora  mammillaria  and  the  oculomotor  nerve  (Oppenheim) . .  33 

16.  Heubner's  arterial  disease.    Infiltration  of  the  adventitia,  media,  intima 

(Nonne) 34 

17.  Section  of  a  gumma  from  the  base  of  the  brain  (Nonne) 35 

18.  The  remnants  of  a  vein  (phlebosclerosis)  (Nonne) 36 

19.  Vein  from  a  section  from  meningitis  encephalitis  luetica  (gummosa) 

(Nonne) 37 

20.  Numerous  elastic  rings  in  arteries  (Nonne) 38 

21.  Obliterated  arteries  in  a  case  of  specific  basilar  meningitis  (Nonne).  . .  39 

22.  Small  area  of  softening  in  the  internal  capsule  caused  by  the  specific 

shutting  off  of  the  arteria  fossae  sylvii  (Nonne) 40 

23.  Localized  arteritis  of  Heubner  (Nonne) 41 

24.  Necrosis  of  the  right  frontal  lobe  on  the  base  in  a  case  of  endarteritis 

luetica  (Schaff er) 43 

25.  Disease  of  an  artery  in  tubercular  leptomeningitis  (Nonne) 45 

26.  Diseased  blood-vessels  in  tubercular  encephalitis  (Nonne) 46 

27.  Spirochsetes  in  the  nerve  bundle,  in  an  initial  lesion  of  the  prepuce 

(Ehrmann) 55 

28.  Arteries  of  the  brain-base  (Monakow) 64 

29.  Arterial  ramification  of  the  peduncular  region  of  the  brain  (Shiamura) .  65 

30.  Transverse  section  through  a  complete  gummatous  degeneration,  and 

greatly  thickened  trunk  of  the  oculomotorius  (Uhthoff ) 98 

31 .  Transverse  section  through  the  base  of  the  brain  just  in  front  of  the  optic 

chiasm  (Uhthoff) 98 

xxi 


xxii  ILLUSTRATIONS 

32.  Transverse  section  through  the  intracranial  optic  trunk,  perineuritis 

and  neuritis  gummosa  (Uhthoff) 99 

33.  Cross  section  of  the  chiasm  not  far  from  the  anterior  angle,  gumma- 

tous  proliferations,  endarteritis  (Oppenheim) Ill 

34.  Scheme  of  the  oculomotor  and  trochlear  nuclei  (Perlia) 117 

35.  Plan  of  the  blood  supply  of  the  brain-stem,  oculomotor  nucleus,  and 

root  areas  (Rossolimo-Shiamura) 118 

36.  Cerebral  basilar  lues.     Rapid  improvement  under  mixed  treatment 

(Nonne) 121 

37.  Isolated  ptosis  in  a  syphilitic  (Nonne) 130 

38.  The  blood  supply  of  the  spinal  cord  in  a  transverse  section  (Dana) ....  209 
39-40.  The  meningitic  proliferation  is  more  strongly  developed  on  the 

posterior  surface  of  the  cord  than  on  the  anterior  (chronic  specific 

basilar  meningitis)  (Bottiger) 212 

41-47.  Inflammatory  thickening  of  the  meninges  (Siemerling) 212 

48.  Part  of  a  transverse  section  from  the  dorsal  side  of  the  cord,  with 

the  adjacent  pia  and  the  root  bundles  lying  in  it  (Siemerling) 213 

49.  Thrombosis  of  diseased  vessels,   with  secondary  hemorrhages   (Wil- 

liamson)    214 

50.  Intraspinal  gummata  with  surrounding  meningitis  (Williamson) 215 

51-53.  Gliosis  and  peripheral  sclerosis  as  a  result  of  deficient  blood  supply 

in  the  periphery  of  the  spinal  cord  caused  by  arterial  disease  in  the 
meninges  (Nonne) 217 

54.  Blood  supply  in  a  transverse  section  of  the  spinal  cord  (Williamson) .  .  225 

55.  Syphilitic  spinal  meningitis  (Homen) 226 

56-59.  Myelitis  acuta  lumbalis  syphilitica  (Nonne) 239 

60-64.  Negative  findings  in  a  clinically  acute  transverse  myelitis 245 

65.  Poliomyelitis  anterior  chrdnica,  choroiditis  luetica  (Nonne) 246 

66.  Poliomyelitis    anterior   chronica    (lues   previously).      The   upper   ex- 

tremities are  completely  paralyzed  and  atrophic  (Nonne) 247 

67-68.  Meningomyelitis  chronica  syphilitica  (Nonne) 249 

69-72.  The  influence  of  therapy  in  a  genuine  Brown-Sequard  symptom- 
complex  (Nonne) .  251 

73-78.  Spastic   paraplegia  inferior,    chronic   dorsal   myelitis,  with   incom- 
plete Brown-Sequard  (Nonne) 253 

79-83.  Combination  of  incipient  tabes  with  chronic  dorsal  myelitis  (Brown- 
Sequard)  (Nonne) 253 

84-90.  Two  cases  of  combined  system  disease  in  luetics  (Nonne) 254 

91.     Scheme  of  the  blood  supply,  transverse  section  of  the  spinal  cord 255 

92-93.  Pseudotabes  syphilitica  (Eisenlohr) 276 

94.  Diffuse  sarcomatous  infiltration  of  the  pia  mater  (middle  dorsal  re- 

gion) (Nonne) 297 

95.  Diffuse  sarcomatous  infiltration  of  the  pia  mater  (Nonne) 297 

96.  Primary  syphilitic  root  neuritis  (Buttersack) 300 

97.  Scheme  for  the  indication  of  mercurial  treatment  in  tabes  (Minor) ....  374 

98.  Hauptmann's  apparatus  for  intravenous  salvarsan  injections 378 


SYPHILIS 

AND 

THE  NERVOUS  SYSTEM 


The  Importance  of  a  Knowledge  of  Syphilis  of  the  Nervous 
System. — We  are  going  to  deal  in  the  following  chapters 
with  the  theme  "syphilis  and  the  nervous  system."  The 
subject  is  a  very  important  one.  In  treating  it,  it  will  be 
necessary  to  consider  almost  the  entire  pathology  of  the 
nervous  system.  Much,  corresponding  to  its  importance, 
must  be  dealt  with  thoroughly,  while  some  it  will  only  be 
necessary  to  touch  briefly  in  passing.  A  knowledge  of  this 
chapter  of  internal  medicine  is  of  great  importance  to 
every  physician.  The  prophylaxis  and  therapy  open  up 
a  wide  and  often  thankworthy  field,  while,  on  the  other  hand, 
a  failure  to  recognize  the  specific  nature  of  a  disease  brings 
with  it  frequently  far-reaching  and  disastrous  results. 

The  Increase  of  Syphilis. — Is  syphilis  of  the  nervous  sys- 
tem of  frequent  occurrence?  The  voluminous  literature 
pertaining  to  this  subject  would  lead  us  to  presume  that  it 
was.  Particularly  the  great  increase  of  this  literature  in 
the  last  decade  would  cause  one  to  think  syphilis  to  be  of 
more  frequent  occurrence  than  formerly.  The  hospital  sta- 
tistics on  this  point  do  not  prove  the  contention.  They 
merely  indicate  the  greater  tendency,  on  the  part  of  the 
laity,  toward  hospital  care,  and  for  this  reason  more  access- 
ible information  from  hospital  records.  Reliable  statistical 
knowledge  of  the  distribution  of  syphilis  is  lacking.  It  can 
only  be  obtained  when  the  physician  is  compelled  to  report 
such  cases  in  the  same  manner  as  he  does  other  infectious 

diseases. 

i 


2  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

During  a  journey  in  Montenegro,  three  years  ago,  it 
was  very  interesting  to  me  to  learn,  from  a  physician  of 
the  country,  that  every  syphilitic  who  presented  himself 
to  a  physician  for  treatment  must  remain  under  medical 
control  for  a  period  of  three  years.  If  he  withdrew  from 
this  supervision,  upon  command  of  the  reigning  prince  he 
was  interned  for  a  period  of  three  years,  or  until  the 
syphilis  in  its  infectious  stage  was  considered  to  have 
terminated. 

In  a  land  where  absolutism  does  not  exist,  and  where  the 
population  is  large,  such  means  would  not  be  feasible. 
Denmark,  Norway,  and  Sweden  are  to  my  knowledge  the 
only  European  countries  which  require  a  notification,  on 
the  part  of  the  physicians,  of  sexual  diseases.  All  cases  of 
sexual  disease  there,  without  mention  of  the  name,  must 
be  reported  weekly  to  the  government  statistical  office. 

From  the  standpoint  of  reason,  however,  we  can  easily 
comprehend  how  syphilis  might  be  more  prevalent  at  the 
present  time  than  formerly.  It  is  a  disease  which  is  trans- 
mitted from  person  to  person,  depending  to  a  large  extent 
on  human  intercourse,  which  in  the  entire  world  has  been 
greatly  increased.  More  difficult  conditions  of  life  have 
also,  from  the  poorer  classes,  driven  more  women  to  prosti- 
tution and  withheld  the  men  from  entering  into  the  marital 
state. 

Increase  of  Nervous  Disease  in  General. — That  nervous  dis- 
ease in  general  has  been  on  the  increase  is  a  well-recognized 
fact.  The  causes  for  this  are  various.  The  development  of 
Germany  into  an  industrial  nation  has  caused  a  great  in- 
crease in  the  proletariat,  not  including  in  this  class  the 
skilled  workmen,  but  only  the  under  laborer  and  the  poorly 
paid  laborer  living  in  the  poorer  quarters  of  our  large 
cities.  One  should  also  mention  here  the  great  army  of 
nervous  people  who  have  been  created  by  the  accident  in- 
surance laws.  Every  physician  who  comes  in  contact  with 
these  cases  knows  that  often  these  accidents  form  the  start- 
ing point  for  different  kinds  of  nervousness,  and  in  the 
majority  of  cases,  not  the  injury  itself  nor  the  series  of 
nervous  shocks  which  may  surround  it  are  responsible  for 


INTRODUCTION  3 

the  resulting  symptoms,  but  the  fight  for  the  insurance 
allowance  makes,  nowadays,  the  injured  workman  a  nervous 
cripple.  We  may  assume  with  reference  to  our  theme  a 
priori  that  in  general  a  weaker  nervous  system  is  being 
subjected  to  a  greater  strain. 

Rudolph  Virchow  in  1858,  in  his  classical  studies  con- 
cerning the  nature  of  constitutional  syphilitic  affections, 
came  to  the  conclusion  that  syphilis  in  the  body  attacks  by 
preference  a  locus  minoris  resistentice.  Later  investiga- 
tions into  this  subject  have  only  confirmed  Virchow 's 
deductions. 

Frequency  of  Syphilitic  Disease  of  the  Nervous  System. — In 
order  to  determine  the  frequency  of  syphilitic  disease  of 
the  nervous  system,  one  can  make  use  of  the  statistics  of 
practising  neurologists.  From  1892  to  1901  I  have  exam- 
ined out  of  my  private  practice  5500  persons  with  nervous 
disease.  From  this  number  specific  disease  of  the  nervous 
system  was  diagnosed  85  times.  Of  other  organic  nervous 
diseases,  there  were  84  cases  of  tumor  cerebri  and  38  cases 
of  multiple  sclerosis.  The  review  of  the  material  at  Eppen- 
dorf Hospital  during  the  same  ten  years  showed  that  from 
72,180  patients,  who  were  received  into  the  hospital  during 
this  period,  the  diagnosis  of  nervous  syphilis  was  made 
282  times.  Cases  of  tabes  and  dementia  paralytica  were 
not  included  in  this  list. 

The  objection  that  perhaps  many  cases  of  nervous 
syphilis  had  sailed  under  other  flags  and  were  placed  in 
other  categories  is  scarcely  valid  when  we  consider  the 
character  of  the  men  who  were  working  at  Eppendorf 
during  this  time.  They  were  such  men  as  Eisenlohr,  Kast, 
and  above  all  Rumpf,  whose  book,  "  Syphilitic  Diseases  of 
the  Nervous  System,"  published  in  1887,  has  been  a  land- 
mark on  the  pathway  to  a  better  knowledge  of  nervous 
syphilis. 

From  1903  to  1907,  out  of  5649  nervous  patients  seen  in 
my  private  practice,  I  diagnosed  nervous  syphilis  88  times. 
During  the  same  five  years  I  saw  42  cases  of  brain  tumor 
and  45  cases  of  multiple  sclerosis.  From  1903  to  1907  at 
my  clinic  at  the  Eppendorf  Hospital  I  treated  9936  cases 


4  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

of  nervous  disease  and  made  the  diagnosis  of  nervous 
syphilis  104  times.  From  my  private  cases  from  1892  to 
1901  the  percentage  of  patients  with  nervous  syphilis 
amounted  to  1.5.  At  the  same  time  from  the  entire  number 
of  patients  received  at  the  medical  clinic  at  Eppendorf,  the 
percentage  was  0.4.  During  the  last  five  years,  from  1903 
to  1907,  from  my  private  clinic  the  percentage  was  1.5. 
At  my  clinic  at  the  Eppendorf  Hospital  the  percentage  was 
1.  In  recent  years  because  of  referred  patients  this  per- 
centage has  been  greatly  increased. 

One  can  see  from  these  statistics  that  while  nervous 
syphilis  cannot  be  regarded  as  a  relatively  frequent  ner- 
vous disease,  yet  it  occurs  about  twice  as  often  as  tumor 
cerebri  and  multiple  sclerosis.  Hjelmann  states  as  his 
opinion  that  brain  syphilis  develops  in  from  15  to  25  of 
every  1000  cases  of  syphilis.  Keumont  in  his  book,  l  i  Syph- 
ilis and  Tabes  Dorsalis, "  from  3400  cases  of  syphilis  found 
290  with  syphilis  of  the  nervous  system.  Engelstedt  and 
Gerhardt  have  come  to  the  same  conclusions. 

Historical. — The  history  of  syphilis  of  the  nervous  sys- 
tem began  shortly  after  the  first  great  epidemic  of  syphilis 
at  the  end  of  the  fifteenth  century.  It  was  a  well-known  fact 
that  the  disease  could  affect  the  internal  organs  and  that 
paralysis  might  result  from  it.  However,  John  Hunter, 
in  1790,  taught,  with  the  entire  weight  of  his  authority,  that 
the  internal  organs  were  never  affected  by  the  disease. 
Ulrich  von  Hutten  recognized  that  paralysis  occurred  in 
some  cases  among  syphilitics,  but  he  attributed  this  to  the 
use  of  mercury. 

As  disease  of  the  bones  of  the  cranium  not  infrequently 
occurred,  it  was  natural  that  this  was  often  ascribed  as  the 
cause  of  the  paralysis  and  other  symptoms  of  the  brain 
irritation.  Lallemand  first,  in  1834,  demonstrated  con- 
clusively that  syphilitic  disease  of  the  meninges  and  brain 
substance  did  occur.  The  studies  of  Griesinger,  Esmarch, 
C.  Westphal,  Gros,  Lancereaux,  Wagner,  and  Wunderlich 
have  also  contributed  greatly  to  the  establishment  of  the 
relationship  between  syphilis  and  brain  affections. 

Gummatous  Processes  and  Heubner's  Arteritis. — Our  pres- 


INTRODUCTION  5 

ent  knowledge  of  syphilis  became  possible  only  after  two 
discoveries  with  reference  to  its  nature  had  been  made. 
The  first  was  the  description  of  the  gummatous  character 
of  specific  processes,  by  Virchow,  and  the  other  was  the 
disease  of  the  cerebral  arteries  due  to  syphilis,  described 
by  Heubner.  These  two  scientists  have  enriched  this  par- 
ticular field  as  no  others  have  done,  because  their  dis- 
coveries first  brought  clear  pathological  anatomical  concep- 
tions, where  before  everything  was  uncertain  and  chaotic. 

The  Symptoms  in  Themselves  Not  Characteristic. — The  first 
clinical  observations  concerning  nervous  syphilis  appeared 
to  give  to  their  character  something  of  a  similarity,  a  more 
or  less  definite  symptom  complex.  However,  this  view  was 
opposed  by  many  experienced  authors.  Gowers  was  one  of 
the  first  to  emphasize  the  fact  that  syphilis  of  the  nervous 
system  produced  no  symptoms  or  combination  of  symptoms, 
which  might  not  be  called  forth  by  other  causes.  We  will 
by  the  aid  of  individual  clinical  pictures  often  have  the 
opportunity  of  referring  to  the  correctness  of  this  view. 
We  will  be  able  to  observe  that  tumors  of  the  brain  and 
spinal  cord  as  well  as  multiple  sclerosis  can,  for  a  long 
time,  present  clinical  symptoms  which  bear  a  great  simi- 
larity to  specific  disease  of  the  nervous  system.  One  may 
also  add  that  this  resemblance  extends  as  well  to  the  patho- 
logical examination.  This  fact  is  particularly  well  exem- 
plified in  many  cases  of  tubercular  disease  of  the  central 
nervous  system,  especially  since  our  knowledge  concerning 
tuberculosis  of  the  spinal  cord  has  been  extended. 

The  Infectious  Factor. — We  know  that  it  is  not  always 
possible  to  differentiate  macroscopically  or  microscopically 
whether  we  have  in  the  existing  case  a  retrograde  changed 
tubercle  or  a  gummatous  nodule,  whether  an  induration 
is  the  beginning  of  a  tubercular  or  syphilitic  process.  It 
not  infrequently  happens  that  both  syphilis  and  tubercu- 
losis may  exist  in  a  state  of  isolation  in  an  organ,  and  this 
is  true  with  regard  to  the  nervous  system.  Sarcoma  of 
the  spinal  cord  may  also  present  similar  symptoms  of 
specific  disease.  These  difficulties  have  long  been  recog- 
nized, and  for  this  reason  the  search  for  the  cause  of  the 


6  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

infection  in  syphilis  had  a  practical  purpose.  The  real  his- 
tory of  these  endeavors  had  its  beginning  in  1879.  In  this 
year  Klebs  communicated  his  experimental  results  which 
had  been  obtained  from  working  with  monkeys.  His  con- 
clusions were: 

First.  That  human  syphilis  may  be  communicated  by 
vaccination  with  newly  formed  syphilitic  tissue  to  animals. 
In  infected  monkeys  exactly  similar  processes  occur  as  in 
human  beings. 

Second.  In  the  specific  processes  of  humans  are  found 
certain  low  vegetable  organisms,  fission  fungi,  which  grow 
outside  of  the  body,  and  take  on  peculiar  and  characteristic 
forms,  which  he  calls  l  '  Helicomonaden. " 

Third.  Through  the  transmission  of  these  peculiar 
forms  to  suitable  animals,  changes  can  be  produced  which 
correspond  to  those  occurring  in  human  primary  syphilis 
as  well  as  to  those  brought  about  through  vaccination  in  the 
monkey. 

From  this  time  forth  work  was  done  to  discover  the 
exciting  cause  of  syphilis.  The  difficulties  in  the  way  of 
obtaining  definite  results  were  due  chiefly  to  three  things: 
(1)  a  lack  of  familiarity  with  the  numerous  non-pathogenic 
bacteria  found  in  both  the  male  and  female  genitalia;  (2) 
to  the  fact  that  one  nowadays  cannot  experiment  on  man; 
and  (3)  in  the  animal  experiments  hitherto  the  fact  opposed 
that  human  syphilis  was  supposed  not  to  be  transmissible 
to  animals. 

The  Transmission  of  Syphilis  to  Monkeys. — An  entirely 
new  era  appeared  in  the  investigation  on  this  subject  when, 
Metschnikoff  and  Eoux  in  Paris,  and  also  A.  Neisser,  had 
succeeded  in  a  manner  entirely  free  from  objection  in  trans- 
mitting syphilis  to  monkeys. 

Metschnikoff 's  article,  delivered  at  the  International 
Dermatological  Congress  in  Berlin  in  1904,  as  well  as 
Neisser 's  communication  entitled  "My  Attempts  in  the 
Transmission  of  Syphilis  to  Monkeys,"  acted  as  an  in- 
spiration. Metschnikoff  reported  out  of  nine  chimpanzees 
infected  with  syphilitic  virus  nine  developed  syphilis. 

Schaudinn's  Spirochaete  pallida. — The  much  sought  for  goal 


INTRODUCTION  7 

of  discovering  the  exciting  cause  of  syphilis  was  left  to  a 
non-medical  man  to  attain.  The  zoologist  Schaudinn  was 
the  first  to  see  the  Spirochcete  pallida  unstained,  and  in 
them  to  recognize  the  exciting  cause  of  syphilis.  Not 
through  a  lucky  chance,  but  as  a  result  of  his  studies  con- 
cerning spirochaete,  he  was  consistently  brought  to  his 
epoch-making  discovery. 

His  first  communication  concerning  the  spirochaete  he 
published  together  with  Hoffman  in  1905.  These  two  scien- 
tists reported  that  they  had  found  true  spirochaete,  not 
only  superficially  in  primary  lesions  and  papules,  but  also, 
which  is  of  especial  interest,  deep  in  the  substance  of  these 
lesions.  The  first  confirmation  of  this  discovery  jeame  a 
week  later  through  E.  Paschin,  of  Hamburg,  who  stained 
old  preparations  from  three  primary  lesions  with  Giemsa's 
solution  and  was  able  to  demonstrate  the  spirochaete  in  all 
of  them. 

At  the  present  time  the  spirochaetes  have  been  found  in 
secretions  from  the  primary  lesion  as  well  as  in  all  second- 
ary lesions,  in  the  lymph-glands,  in  all  the  internal  organs, 
in  the  blood-vessels,  in  the  blood  itself,  and  particularly 
numerous  in  syphilitic  foetuses. 

What  concerns  our  special  theme  is  that  previous  to 
1910  *  the  spirochaete  had  only  been  found  in  the  brain  and 
spinal  cord  in  syphilitic  foetuses.  Banke  exhibited  at  Baden 
Baden  at  a  meeting  of  the  Southwestern  German  Neurolo- 
gists preparations  of  the  pia  mater  and  the  blood-vessels 
of  the  brain  cortex  taken  from  a  child  with  hereditary 
.syphilis  in  which  the  spirochaetes  were  to  be  seen.  Stras- 
mann  in  1910  reported  a  case  of  a  cerebral  gummatous 
tumor  in  which  he  found  the  Spirochtete  pallida. 

Proof  of  Specific  Infection  and  Its  Difficulties. — Personal 
confirmation  of  the  specific  infection  still  remains  important 
for  the  diagnosis.  The  personal  proof  of  a  past  syphilis 

*  Since  1910  the  spirochaete  has  been  demonstrated  in  other  tissues  of 
the  central  nervous  system  and  in  the  spinal  fluid  in  cases  of  nervous 
syphilis.  Just  recently  Noguchi  has  found  the  spirochsete  in  forty-eight 
brains  out  of  two  hundred  examined  in  cases  of  general  paresis  and  in  the 
spinal  cord  in  one  case  out  of  twelve  examined  in  tabes,  deeply  embedded 
in  the  nervous  tissue.  (Journal  of  Experimental  Science,  Feb.  1,  1913; 
Miinchener  med.  Wochenschrift,  Apr.  8,  1913.) 


8  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

has  often  to  battle  with  the  greatest  difficulties.  We  must 
consider  first  that  many  persons  against  their  better  knowl- 
edge will  deny  a  past  infection,  and,  second,  a  number  of 
persons  unfortunately  do  not  know  that  at  some  earlier 
period  they  have  received  an  infection. 

The  securing  of  an  admission  of  a  previous  infection 
depends  very  much  upon  the  manner  in  which  the  physician 
approaches  his  patient  and  the  persistence  of  his  question- 
ing. The  conscious  denial  of  an  earlier  infection  from  a 
patient  who  thoroughly  understands  the  importance  of  a 
truthful  answer,  according  to  my  experience,  seldom  occurs. 
There  is  no  social  position,  no  age  in  which  one  can  exclude 
the  possibility  of  a  previous  infection.  One  must  think  of 
the  possibility  of  an  extramatrimonial  infection,  which  is  as 
apt  to  occur  among  the  higher  classes  as  among  the  lower, 
and  which  is  not  infrequently  denied. 

Ignorance  of  the  Infection. — With  regard  to  those  who 
are  absolutely  unaware  of  ever  having  had  an  infection, 
Fournier  says  that  in  50  per  cent,  of  his  cases  in  women 
he  found  this  to  be  true.  He  speaks  of  the  frequency  of 
"syphilis  insontium."  Fleiner  some  years  ago  reported  a 
series  of  cases  of  severe  tertiary  lues  in  patients  who  had 
no  knowledge  of  either  the  primary  infection  or  of  ever 
having  had  any  secondary  symptoms.  Hirschl  has  re- 
ported from  Lang's  clinic  in  Vienna  63  cases  of  late  forms 
in  syphilis,  in  which  there  was  absolute  knowledge  of  a 
previous  infection  in  the  personal  history  of  54  per  cent, 
of  the  cases,  in  9.5  per  cent,  a  probable  knowledge,  and  in 
36.5  per  cent,  of  the  cases  there  was  in  the  personal  history 
no  evidence  whatever. 

Extragenital  Infection. — It  happens  quite  often  with  those 
infected  extragenitally  that  they  have  no  knowledge  of  the 
infection,  particularly  where  the  infection  does  not  occur 
in  a  prominent  situation,  as  the  lip  or  face,  but  on  the 
mammae,  or,  for  example,  the  fingers.  Hahn,  out  of  the 
large  clinical  material  of  Engel-Beimers  at  Hamburg  at  his 
disposal,  estimated  the  frequency  of  extragenital  infection 
in  Germany,  France,  and  Denmark  as  varying  between  4.5 
per  cent,  and  5.5  per  cent.  Von  Beloussow  states  that  in 


INTRODUCTION  9 

Russia  the  number  of  genitally  infected  is  only  from  22  to 
26  per  cent,  of  the  total  number  of  infections. 

I  am  able  to  report  some  cases  out  of  my  own  experience 
illustrative  of  extragenital  infection. 

CASE  ONE. — A  coachman  whom  I  treated  for  tabes  dor- 
salis  had,  when  a  boy  thirteen  years  old,  slept  for  many 
consecutive  nights  in  the  same  bed  with  a  farm  hand.  Two 
months  later  he  was  treated  in  the  hospital  at  Biitzow  for  a 
syphilitic  eruption  on  the  buttocks. 

CASE  Two. — A  strong,  healthy  woman  came  to  me  with 
symptoms  of  a  beginning  aneurism  of  the  aorta.  After  a 
thorough  examination  the  following  history  was  obtained: 
Before  her  first  marriage  she  was  engaged,  and  from  her 
betrothed  was  infected  on  the  lip,  subsequently  under- 
going an  inunction  cure. 

Cases  of  extragenital  infection  at  an  early  age  in 
patients  with  tabes  and  general  paralysis  are  also  reported 
by  Kron,  von  Bad,  Halban,  Kutner  and  others.  Bradshaw's 
case  might  be  related  here. 

A  man  40  years  old,  with  tabes,  had  acquired  his  syphilis 
18  years  before.  At  that  time  he  suffered  with  chapped 
hands,  and  had  as  a  room-mate  a  man  who  had  a  syphilitic 
ulcer  on  the  back  of  one  of  his  hands. 

Syphilitic  Findings  Postmortem  with  Negative  History. — 
In  my  department  at  the  Eppendorf  Hospital  during  1897 
and  1898  every  new  patient  received  was  questioned  care- 
fully for  a  history  of  syphilis,  and  their  bodies  subjected 
to  a  painstaking  examination  for  evidences  of  a  previous 
lues.  In  a  number  of  cases,  in  which  the  history  and  ex- 
amination were  negative,  postmortem  visceral  syphilis  was 
found. 

A  review  of  the  literature  of  cases  of  nervous  syphilis 
teaches  us  that  many  cases  of  a  specific  nature  are  found 
where  the  history  of  an  infection  is  wholly  lacking.  One 
could  report  almost  indefinitely  observations  of  this  charac- 
ter. Such  examinations  warn  us  that  we  must  be  very 
careful  of  the  use  we  employ  of  a  denial  with  reference  to 
a  specific  infection. 

One  should  also  use  every  means  in  cases  of  doubtful 


10  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

diagnosis,  where  previous  syphilis  is  admitted,  to  assure 
themselves  of  the  correctness  of  the  admission.  The  laity 
are  sometimes  inclined  to  regard  every  infection  of  the 
genitalia,  such  as  gonorrhea,  soft  chancre  and  even  herpes, 
as  specific  in  nature.  Dualism  of  the  soft  and  hard  chancre, 
since  the  discovery  of  the  Spiroclicete  pallida,  seems  to  be 
established  beyond  any  doubt.  On  the  other  hand,  one  must 
remember  that  in  certain  rare  cases  the  specific  primary 
lesion  may  constitute  the  only  evidence  of  syphilitic  infec- 
tion, no  other  symptoms  appearing. 

Examination  for  Past  Evidence  of  Syphilis. — If  one  has  had 
the  opportunity  of  being  stationed  for  some  time  in  the 
women's  department  of  a  large  syphilitic  clinic,  one  will 
soon  recognize  how  unobtrusive,  hidden  and  apparently 
harmless  an  affection  the  primary  lesion  often  is.  The 
"erosion  chancreuse,"  a  little  elevation,  an  insignificant 
induration,  is  all  there  is  to  be  seen.  The  glandular  swell- 
ing in  the  groin  in  women  who  $0  no  manual  work  may 
be  very  slight,  and  can  be  entirely  lacking.  The  secondary 
symptoms  also  may  be  so  little  manifest  that  the  physician 
must  make  a  thorough  search  in  order  to  discover  them. 
One  can  easily  understand  how  women  so  often,  even  with 
the  best  intentions,  are  unable  to  give  any  positive  infor- 
mation. 

Very  often  a  careful  examination  of  the  entire  body  will 
disclose  evidences  of  a  previous  infection.  The  more  thor- 
oughly this  examination  is  performed,  the  greater  one's  ex- 
perience with  the  superficial  manifestations  of  syphilis  on 
the  skin,  the  mucous  membrane  and  bones,  the  sooner  one 
will  be  able  to  form  a  positive  opinion.  Pay  particular 
attention  to  the  pigment-syphilide,  that  is,  the  residual  in 
the  skin  and  mucous  membrane  from  specific  efflorescence, 
also  alopecia  and  leucoderma,  especially  the  latter,  which 
one,  in  spite  of  the  fact  that  Lewin  has  demonstrated  that  it 
may  occur  in  non-luetics,  should  regard  as  a  most  important 
indication  of  a  past  syphilis.  Palmer  in  600  cases  found 
it  present  in  70  per  cent. 

Give  heed  to  chlorosis,  the  lattice-like  disruption  of  the 
tonsils,  the  presence  of  struma,  the  significance  of  which  as 


INTRODUCTION  11 

symptoms  of  earlier  syphilis  Engel-Reimers,  of  Hamburg, 
has  especially  pointed  out.  The  reproach  that  every  scar 
and  pigmented  spot  is  often  regarded  as  evidence  of  a  past 
syphilis  is  not  valid  when  applied  to  the  intelligent  and 
honest  observer. 

There  is  always,  however,  an  inconsiderable  number  of 
cases,  even  during  the  infectious  period,  which  after  the 
most  painstaking  examination  present  no  evidence  of  lues. 

Palmer,  under  the  direction  of  Engel-Reimers,  demon- 
strated in  600  women  whom  I  had  treated  during  the  initial 
lesion,  and  some  of  them  during  the  secondary  symptoms 
also,  that  a  few  months  later  the  most  careful  physical 
examination  failed  to  disclose  the  slightest  stigma  of 
syphilis.  In  such  cases  possibly  Wassermann's  serum  reac- 
tion will  aid  in  the  diagnosis.  In  a  later  chapter  we  will 
see  that  the  Wassermann  reaction  occurs  frequently, 
although  by  no  means  regularly,  in  those  who  either  have 
or  have  had  syphilis.  In  the  proper  estimation  of  the  re- 
sults of  the  serum  reaction  great  caution  is  necessary.  This 
subject,  however,  will  be  discussed  in  another  chapter. 

I  wish  to  emphasize  particularly  that  a  large  number 
of  healthy  children  is  no  contradiction  of  a  past  lues  in  the 
parents.  I  have  repeatedly  encountered  this  idea  even 
among  experienced  physicians. 

Not  Every  Organic  Nervous  Disease  in  a  Person  Infected 
with  Syphilis  is  of  Specific  Origin. — CASE  THREE. — For  exam- 
ple: A  man  thirty-eight  years  old  acquired  syphilis  from 
a  razor  cut  on  the  chin.  Two  years  later  he  presented  him- 
self to  me  with  severe  radiating  pains  and  a  spastic  para- 
plegia of  the  lower  extremities  first,  and  then  later  the 
upper  extremities,  for  which  there  appeared  to  be  no  other 
attributable  cause.  Under  an  energetic  antispecific  treat- 
ment, with  pauses,  the  severe  disturbance  of  the  spinal  cord 
disappeared  in  the  course  of  a  year  and  a  half.  Four  years 
later  the  patient,  in  whom  during  the  two  years  previous 
to  his  death  tubercular  disease  of  the  vertebrae  had  devel- 
oped with  multiple  burrowing  abscesses,  died  with  a  general 
marasmus.  At  the  postmortem  it  was  ascertained  that  in 
addition  to  a  fresh  caries  of  the  vertebrae  there  were  scars 


12 

of  an  old  tubercular  process  in  the  cervical  and  lower  dorsal 
regions  where  the  spinal  cord  had  been  compressed  in  two 
places.  There  was  no  other  evidence  of  either  tuberculosis 
or  syphilis  in  the  body. 

There  May  Also  be  a  Combination  of  Several  Etiological 
Factors. — Along  with  a  proven  syphilis  there  may  exist  a 
chronic  alcoholism,  a  head  injury  or  an  acute  infectious 
disease,  which  may,  either  directly  or  indirectly,  affect  the 
nervous  system. 

One  must  not  neglect  in  their  etiological  reflections 
alcohol.  We  know  since  Bourdon's  work  in  1862  that  the 
pseudotabes  alcoholica  may  sometimes  very  closely  re- 
semble disease  of  the  spinal  cord,  that  alcohol  in  the  etiology 
of  progressive  paralysis,  as  well  as  of  epilepsy,  together 
with  syphilis,  plays  an  important  part.  Because  of  the  fre- 
quent combination  of  syphilis  and  alcoholism  we  often  do 
not  know  in  an  apoplexy  which  factor  to  ascribe  the  brain 
hemorrhage  to. 

If  a  patient  with  the  history  of  an  earlier  syphilis  has 
reached  an  age  where  arteriosclerosis  may  be  expected  to 
exist,  the  assumption  of  a  relationship  between  the  former 
lues  and  the  apoplexy  loses  greatly  in  probability. 

Moreover  chronic  alcoholism  has  an  especial  importance 
since  it  weakens  the  nervous  system,  and  in  those  persons 
who  have  been  subjected  to  the  toxin  of  lues  makes  it  more 
easily  susceptible  to  disease.  Oppenheim  states  that  ex- 
cesses in  Bacco  in  non-alcoholics  may  give  the  impulse  for 
the  beginning  of  brain  syphilis. 

It  has  long  been  recognized  that  an  injury  to  the  head 
in  luetics  can  be  the  cause  of  an  outbreak  of  brain  syphilis. 
Especially  has  this  been  observed  in  the  development  of 
brain  gummata.  Henneberg  mentions  a  case  anatomically 
proven  of  meningitis  basalis  luetica  in  a  previously  entirely 
healthy  man  produced  by  a  severe  fall  on  the  head. 

The  Diagnostic  Importance  of  Antispecific  Therapy. — The 
assumption  of  the  specific  nature  of  a  nervous  disease  be- 
cause it  disappears  under  antispecific  treatment  is  in  the 
main  valid,  but  there  may  be  exceptions,  as  the  case  cited 
with  tuberculosis  of  the  vertebrae  and  compression  of  the 


INTRODUCTION  13 

spinal  cord,  which  apparently  receded  under  the  antispecific 
treatment,  shows. 

On  the  other  hand,  as  Fournier,  Lancereaux,  Heubner, 
and  Rumpf  have  especially  called  attention  to,  there  are 
specific  gummatous  processes  which  not  infrequently  are 
refractory  to  mercury  and  potassium  iodid.  Gowers  and 
Horsley  have  stated,  basing  their  position  on  pathological- 
anatomical  examinations,  that  brain  gummata  are  not  cura- 
ble by  mercury  and  iodid,  and  recommend  trepanation  as 
the  indicated  therapy. 

In  two  cases  of  gummatous  character  in  the  brain,  in 
each  one  of  which  the  attending  physician  had  excluded 
syphilis  because  mercury  and  iodid  had  not  benefited,  and 
also  in  my  department  at  Eppendorf,  had  shown  themselves 
to  be  completely  refractory  to  antispecific  treatment,  I  made 
the  postmortem.  In  both  cases  I  was  able  to  demonstrate 
microscopically  the  gummatous  nature  of  the  brain  changes. 

In  many  cases  of  syphilis  of  the  nervous  system  under 
the  influence  of  the  syphilis,  certain  changes  are  produced 
in  the  arteries  which  create  disturbances  in  the  nutrition 
of  the  part  supplied  by  the  arteries  and  lead  to  necroses. 
Such  necroses  are  not  in  themselves  specific  and  one  should 
not  expect  to  influence  them  by  antispecific  therapy.  One 
should  also  consider  that  in  so  far  as  the  function  itself  is 
concerned  at  the  time,  the  result  is  the  same  whether  the 
functionating  nervous  element  is  compressed  by  newly 
formed  syphilitic  tissue  or,  as  a  result  of  the  treatment, 
by  actual  scar  tissue. 

Keeping  this  in  mind  one  can  easily  understand  why 
there  is  only  a  limited  number  of  early  cases  in  which 
from  treatment  we  may  expect  a  full  recovery.  My  experi- 
ence has  been  that  physicians  usually  are  too  optimistic  in 
their  prognosis  of  organic  nervous  affections  recognized 
as  syphilitic.  A  review  of  the  literature  pertaining  to  this 
subject  is  also  open  to  the  same  criticism.  Too  great  an 
influence  is  attributed  to  the  therapeutical  factor. 

Summary. — First.  Disease  of  the  nervous  system  due  to 
syphilis  is  not  infrequent.  It  is  also  probable  that  its  fre- 
quency is  on  the  increase. 


14  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

Second.  iSince  every  clinical  manifestation  of  nervous 
syphilis  may  be  caused  by  non-specific  processes  it  is  ex- 
tremely important,  where  possible,  to  obtain  a  history  of 
syphilis  in  the  patient. 

Third.  When  the  infection  has  once  been  determined, 
that  does  not  prove  that  the  present  existing  nervous  dis- 
ease is  of  a  specific  nature. 

Fourth.  If  the  symptoms  of  the  existing  case  are  such  as 
specific  disease  may  present,  then  the  probability  of  a 
relationship  increases,  particularly  if  other  signs  of  syph- 
ilis are  present  in  the  'patient. 

Fifth.  The  etiological  value  of  a  proven  syphilis  not  in- 
frequently experiences  more  or  less  of  limitation  because 
of  a  combination  of  causative  factors. 

Sixth.  Success  or  non-success  of  antispecific  therapy  is 
only  conditionally  to  be  regarded  as  a  means  of  differential 
diagnosis. 


II 

PATHOLOGY 

THE  pathological  anatomy  of  specific  disease  of  the 
nervous  system  is  deserving  of  thorough  study.  Only  in 
this  way  are  we  able  to  recognize  on  the  one  hand  that 
the  clinical  symptoms  can  and  must  be  manifold,  and  on 
the  other  that  a  certain  group  of  symptoms  appear  with 
especial  frequency.  The  study  of  nervous  syphilis  obtained 
its  incentive  first  from  the  pathology.  That  the  pathology 
has  been  somewhat  backward  is  due  to  the  fact  that  because 
of  a  more  efficient  therapy  fewer  cases  of  nervous  syphilis 
have  come  to  the  postmortem  table  than  of  other  cases  of 
organic  nervous  disease. 

I  can  find  in  a  review  of  the  postmortem  cases  at  the 
Eppendorf  Hospital,  for  the  years  1890-1899,  only  24  cases 
in  which  specific  disease  of  the  nervous  system  was  demon- 
strated. 

Cranium  and  Vertebrae. — In  relation  to  specific  disease 
of  the  bone  we  are  interested  here  only  in  so  far  as  it 
concerns  the  bones  of  the  cranium  and  spinal  vertebrae. 
Syphilis  manifests  itself  in  bones  as  periostitis,  ostitis  and 
as  gummatous  formations.  Periostitis  occurs  rarely  in  the 
secondary  stage  and  then  only  in  a  moderate  degree.  It 
occurs  much  more  often  in  the  tertiary  stage,  and  results 
frequently  in  this  stage  in  a  wide-spread  and  diffuse 
hyperostosis. 

Along  with  and  following  the  periostitis  an  ostitis  fre- 
quently develops  which  leads  to  the  deposit  of  a  newly 
formed  bony  substance  in  the  medullary  canals,  to  an  ebur- 
neating  osteomyelitis.  The  exostoses  are,  with  the  excep- 
tion of  the  tibia,  most  frequently  located  on  the  cranium. 
The  specific  ostitis,  in  addition  to  causing  hyperostosis, 
may  lead  to  osteoporosis  and  rarefaction  (dry  caries, 
according  to  Virchow).  Necrosis  also  occurs,  due  to  the 
eburnization  with  secondary  blocking  of  the  Haversian 

15 


16  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

canals.  In  this  way  there  occur  more  or  less  extensive  de- 
fects in  the  bones,  and  the  classical  spot  for  these  to  come 
is  on  the  bones  of  the  skull.  Indeed  this  juxtaposition  of 
bony  defects  and  hyperostoses,  which  permanently  remains 
even  after  the  gummatous  tissue  has  been  transformed  into 
connective  tissue,  is  characteristic  of  syphilis  of  the  bones 
and  especially  of  the  bones  of  the  cranium. 

Syphilis  of  the  bones  leads  also,  as  in  all  other  organs 
of  the  body,  to  a  true  gummatous  formation  which  is  tumor- 
like  in  character. 

In  regard  to  the  frequency  of  syphilis  of  the  skull  I  can 
find  only  one  reference.  Mildner,  in  1872,  stated  that  in 
seventeen  cases  of  syphilis  of  the  bones,  the  bones  of  the 
cranium  were  affected  eight  times.  The  disease  occurs  most 
often  in  the  frontal  bone,  along  the  supra-orbital  prom- 
inence ;  then,  in  point  of  frequency,  comes  the  parietal  bone. 
Disease  of  the  bones  in  hereditary  lues  is  of  the  same  char- 
acter as  in  the  acquired  form,  with  the  exception  that  the 
hyperostosis  is  usually  not  so  firm  in  the  hereditary  type 
(Stroebe). 

It  is  clear  that  disease  of  the  bones  may  affect  the  brain 
and  spinal  cord  and  their  membranes,  either  through  pres- 
sure or  by  an  extension  of  the  inflammatory  process  to  the 
nerve  tissue.  However,  one  must  not  consider,  even  in 
extensive  disease  of  the  cranium,  that  affection  of  the  brain 
is  the  rule.  On  the  contrary  one  sees  surprisingly  often, 
even  where  the  brain  is  laid  bare,  that  it  escapes 
involvement.  . 

Syphilis  of  the  Vertebrae.— Specific  disease  of  the  spinal 
column  is  rare.  When  it  occurs  the  cervical  part  seems  to 
be  the  most  often  affected.  In  contrast  to  tuberculosis, 
which  most  frequently  involves  the  dorsal  region,  likewise 
in  contrast  to  tuberculosis,  syphilis  affects  the  spinous  and 
transverse  processes  instead  of  the  bodies  of  the  vertebra. 

There  are  observations  which  demonstrate  the  possibil- 
ity of  severe  late  specific  ulceration  of  the  posterior  wall 
of  the  pharynx  affecting  the  upper  cervical  vertebrae  by  ex- 
tension. In  the  vertebrae  so  attacked  there  comes  a  peri- 
ostitis and  ostitis  and  consequent  necrosis  of  the  bone.  In 


PATHOLOGY  17 

this  manner  the  necrosis  of  the  entire  body  of  a  cervical 
vertebra  originated,  as  well  as  the  casting  off  of  the  necrotic 
and  sequestered  anterior  arch  of  the  atlas  in  the  cases  of 
Autenrieth  and  Fisher.  Gerhardt  mentions  in  his  mono- 
graph concerning  syphilis  of  the  spinal  cord  that  the  spinal 
vertebra?  may  become  affected  by  an  extension  of  the  dis- 
ease from  the  cranial  bones.  In  the  diagnosis  of  a  specific 
lesion  of  the  vertebra?  one  should  consider  carefully  whether 
tubercular  disease  has  not  perhaps  overtaken  a  luetic 
patient.  The  following  observation  in  my  own  experience 
will  illustrate  this : 

CASE  POUR. — A  strong,  healthy  man  became  infected  with 
syphilis.  Two  years  later  he  developed  symptoms  of  cer- 
vical caries  with  secondary  paraplegia  inferior  and  su- 
perior, which  in  the  course  of  a  year,  under  energetic  anti- 
specific  treatment,  disappeared;  then  he  developed  tuber- 
culosis of  the  lungs  and  died.  The  section  proved  the  case 
to  have  been  one  of  tubercular  caries  which  had  healed. 

The  extension  of  specific  disease  of  the  vertebra?  to  the 
spinal  cord  and  its  coverings  from  an  affection  of  the 
vertebra?  occurs  far  less  often  than  an  involvement  of 
the  brain  from  disease  of  the  skull. 

Pathology  of  Nervous  Syphilis. — The  pathological  changes 
which  take  place  in  the  nervous  system  as  a  result  of 
syphilis  can  be  grouped  under  three  great  divisions.  The 
first  is  syphilitic  new  growth,  the  second,  chronic  hyper- 
plastic  inflammation,  and  the  third,  disease  of  the  blood- 
vessels. 

The  results  of  specific  disease  of  the  blood-vessels  are 
not  specific  in  character;  they  are  the  consequences  which 
must  occur  wherever  the  nutrition  of  an  area  is  either  inter- 
fered with  or  suspended.  Necrosis  of  nerve  tissue,  which 
occurs  so  frequently  in  the  brain,  is  only  of  secondary 
origin. 

Besides  the  three  forms  of  specific  disease  of  the  ner- 
vous system  there  occurs  also  a  degenerative  process  of 
varied  type,  which,  for  clinical  reasons,  syphilis  must  be 
regarded  as  standing  in  a  causative  relationship  to.  Patho- 
logically, however,  this  degeneration  is  not  recognized  as 

2 


18 


SYPHILIS  AND  THE  NERVOUS  SYSTEM 


specific.  In  typical  and  atypical  forms  it  is  a  primary 
parenchymatous  degeneration  and  referred  to  under  the 
names  of  post-  or  meta syphilitic  disease.  At  the  outset  it 
may  be  said  that  one  almost  never  finds  one  of  the  three 
types  of  pathological  change  alone,  but  practically  always 
in  combination.  The  division  of  specific  disease  into  pri- 
mary, secondary,  and  tertiary  stages  is  an  old  one. 

It  is  universally  recognized,  at  the  present  time,  that 
the  gummatous  formations  belong  to  the  tertiary  stage. 


Fia.  1. — Cut  from  the  left  paracentral  lobe  (personal  observation).    Under  the  raised  dura,  6, 
a  gummatous  tumor,  a,  which  extends  as  a  diffuse  infiltration  to  part  of  the  brain  cortex,  c. 

Gummata. — Gummata  appear  in  the  form  of  smaller  or 
larger,  single  or  multiple  growths,  or  as  a  diffuse  and  exten- 
sive infiltration. 

Statistics  concerning  the  frequency  of  gummata  have 
only  a  relative  value,  because  many  gummata  under  a  suit- 
able therapy  are  healed,  and  for  this  reason  a  pathological 
diagnosis  is  impossible.  Also,  because  of  their  varied  dis- 
tribution, often  clinically  they  escape  recognition.  Our 
large  material  for  section  at  Eppendorf  (about  2400  cases 
yearly)  shows  that  the  majority  of  cases  of  gummatous 
syphilis  of  the  liver  escape  detection  and  are  diagnosed 
as  nephritis  chronica  or  amyloid  degeneration.  There  are, 
however,  some  statistics  on  this  subject. 

Fournier  found  in  4000  cases  of  late  syphilis,  gummata 
in  6.3  per  cent.,  Gron  in  3471  cases  11.1  per  cent.,  and  again 


PATHOLOGY  19 

in  36,757  specific  cases  gummata  in  11.8  per  cent,  of  the 
cases.  According  to  Gron's  statistics  the  arteries  are  the 
most  favored  location  for  gummata,  of  the  internal  organs 
the  kidneys  and  liver,  and  then  next  in  frequency  comes 
the  brain. 

Gummata  vary  in  size  from  a  hempseed  to  a  walnut. 
However,  many  larger  ones  have  been  described. 


FIG.  2a. — A  hard,  fibrous  gumma  on  the  base  of  the  right  frontal  lobe,  growing  out  from  the  dura. 

(Personal  observation.) 

Thus  Bruns  reports  a  case  in  which  a  gumma  involved 
the  white  substance  of  the  left  occipital  lobe,  the  left  upper 
parietal  lobe,  the  left  gyrus  marginalis  and  angularis  and 
the  posterior  end  of  the  first  two  left  temporal  convolutions. 
Its  color  is  usually  a  grayish  red  in  fresh  specimens.  It 
may  also  take  on  a  yellowish  tinge.  Very  often  it  under- 
goes regressive  changes  and  it  then  appears  yellow.  The 
consistence  of  the  fresh,  not  yet  metamorphosed,  tumor  is 
tough  and  hard;  most  often  it  gives  a  rubber-like  sensation 
to  the  palpating  finger,  which  fact  has  contributed  some- 


20 


SYPHILIS  AND  THE  NERVOUS  SYSTEM 


what  to  its  name.  Through  regressive  changes  gummata 
may  become  soft,  although  usually  only  partially  so.  More 
often  they  become  hard  through  transformation  into  a 
cheesy  mass.  By  mixed  infection  there  may  be  pus 
formation. 

The  favorite  location  is  the  dura,  on  the  convexity  as 
well  as  the  base.    At  the  base  of  the  brain,  gummata  occur 


Fid.  26. — Gummata  on  the  inner  surface  of  the  dura  in  the  right  cranial  convexity. 

particularly  often  as  small  tumors,  with  preference  for 
the  neighborhood  of  the  arterial  trunks.  In  the  substance 
of  the  brain,  gummata  appear  in  the  cortex  and  most  often 
in  the  region  of  the  central  convolutions.  In  this  location 
not  infrequently  they  are  found  infiltrating  the  brain  sub- 
stance so  uniformly  that  the  contour  of  the  cortex  is 
scarcely  changed;  only  its  consistence  becomes  firm  and 
hard.  They  are  sometimes  in  direct  contiguity  with  the 
meninges,  and  when  found  in  the  interior  of  the  brain, 
which  is  not  infrequent,  in  proximity  to  the  large  ganglia. 


PATHOLOGY 


21 


A  man  with  a  specific  infection  developed  symptoms  of 
a  tumor  in  the  left  internal  capsule.  The  postmortem  re- 
vealed a  gummatous  growth  situ- 
ated in  the  left  optic  thalamus  and 
lenticular  nucleus.  In  the  brain 
substance  surrounding  these  gan- 
glia secondary  changes  were  also 
found. 

In  a  case  published  by  me  four 
years  ago  in  which  there  was  a 
combination  of  tabes  dorsalis, 
arteriosclerotic  encephalomalacia 
and  gummi  cerebri,  the  gummatous 
growths  were  located  in  the  right 
lenticular  nucleus  and  the  internal 
capsule,  extending  below  to  the 
optic  tract  and  above  to  the 
nucleus  caudatus. 

In  this  Case  the  WaSSermann  in      FIG.  3o.— Gumma  of  the  dura  (personal 
j-i          i  i         n  T  ...  observation). 

the   blood  was   strongly  positive, 

in  the  spinal  fluid  negative  with  0.4  c.c.  of  fluid  and  strongly 

positive  with  1  c.c. 


FIG.  36. — Gumma  of  the  dura  (personal  observation). 

Grummata  have  been  found  in  the  brain  in  the  frontal, 
parietal,  temporal,  and  occipital  lobes  and  in  the  fourth  ven- 


22 


SYPHILIS  AND  THE  NERVOUS  SYSTEM 


tricle.  They  also  occur  in  the  cerebellum,  pons,  and 
medulla.  Gummatous  nodules  can  develop  in  the  hypoph- 
ysis. How  numerous  they  may  be,  a  cut  taken  from 
Baumgarten  will  illustrate. 

A  beautiful  case  of  gummata  in  the  optic  tract  and  pons 
is  reproduced  from  Cornil  (Fig.  5). 

A  large  pontine  tumor  of  gummatous  origin  is  repre- 
sented in  a  case  of  Lang's  (Fig.  6). 


FIG.  4. — Multiple  gummata  on  the  base  of  the  brain  (Baumgarten,  Virchow's  Archives,  vol.  86). 

As  a  further  example  of  gummatous  growth  on  the  base 
of  the  brain  a  case  of  Siemerling  is  cited.  Observe  in  this 
specimen  the  involvement  of  one  pyramidal  tract  and  the 
interpeduncular  space  (Fig.  7). 

That  in  a  diffuse  disease  of  the  brain,  such  as  general 
paralysis,  disseminated  miliary  gummatous  nodules  do 
occur,  has  been  shown  by  Straussler.  The  following  cut 
taken  from  Straussler 's  work  represents  the  appearance 
of  the  smallest  gummata  under  the  low  and  high  powers  of 
the  microscope  (Figs.  8  and  9). 


PATHOLOGY 


23 


In  Fig.  10  are  shown  by  microphotograph  brain  gum- 
mata  under  the  low  power.    One  can  observe  in  the  centre 

the  necrotic  caseated  tis- 
sue, on  the  periphery  of 
the  same  an  inflammatory 
zone,  within  this  zone  also 
numerous  blood-ve  s  s  e  1  s 
with  thickened  walls. 
Fig.  11  shows  how  the 


FIG.  6. — Syphilis  of  the  central  nervous  ays- 
Fia.  5. — Gummata  of  the  optic  tract  and  pons.  tern  (Traite"  de  Me"decine  2edd.Tome  IX.) 


Th.  opt.        R.N. 


K.  III.    Comm.  p.    Aq.  S. 


.Th.opt. 


24 


SYPHILIS  AND  THE  NERVOUS  SYSTEM 


occurrence  of  fully  sclerosed  blood-vessels  may  indicate 
syphilis  (low  power).  The  elastic  ring  is  all  that  remains 
to  reveal  the  presence  of  vessel  lumina. 

The  surface  of  gummatous  tumors  is  rarely  smooth. 

_ __ ________^___^_____  They  are  mostly  uneven 

I^HMHMO^BH^B^H 

and  nodular.  They  are 
seldom  detached  from 
their  surroundings,  but 
usually  bound  down  by 
inflammatory  adhesions 
to  the  meninges  or  blood- 
vessels. There  is  nearly 
always  an  accompanying 
local  meningitis. 

By  transverse  section 
of  gummi  in  typical 
cases,  one  observes  a 
centrally  situated  yel- 


'   low,    opaque    homogene- 
ous dense  mass,  around 


'     ' »   -.  •'»  JB  WwT»  •    4  >  * 

*  .       •4*-?-*?   '  '.*    /  *        V 

•';>^#:,;:, 

• ,      ,  .  - ;  * ',  t  ?  *•  '.  f~i-jfti 

.   *^j        which  is  a  grayish  semi- 

FIG   8.— Strausaler,  encephalitis  gummoaa.  a,  b,  mill-    transparent  tisSUC,  801216- 
ary  gummata. 

times    dense,    sometimes 

soft  in  consistence.  This  is  again  surrounded,  in  recent 
cases,  by  a  soft  reddish  tissue,  in  old  cases  by  a  dense, 
dry,  often  brownish  substance. 

The  microscopical 
examination  shows  the 
character  of  Virchow's 
granulation  growth. 
Scattered  through  the 
periphery  one  sees 
granulation  cells,  also 
spindle-  and  star- 
shaped  cells  in  the 
connective  tissue  and 

~^ '*'&**'}**$** 


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t*  •••**•  ••<*'./*! 
'    '"?£$ 

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cellular  elements 
which  are  undergoing 
a  fatty  degeneration. 


3. — Encephalitis  gummosa.     Miliary  gummata  with 
giant  cells  (a) . 


PATHOLOGY 


25 


In  the  centre  itself  there  is  usually  only  a  necrotic  cheesy 
mass,  in  which  the  cells  can  no  longer  be  differentiated. 
One  sees  only  a  homogeneous  opaque  mass.  Here  and  there 
one  sometimes  finds,  in  this  part,  giant  cells  and  epithelioid 
cells,  which  cannot  be  distinguished  from  the  giant  cells  so 
commonly  found  in  tuberculosis. 

Giant  cells  were  first  found  in  gummatous  nodules  by 
Jacobson,  then  by  Baumgarten  in  gummata  of  the  testicles 
and  liver,  and  later  by  the  same  scientist  in  gummatous 


FlO.  10. — Gummatous  encephalitis.     (Personal  observation)   (microscopical  examination.) 

nodules  of  the  dura  mater  and  the  adventitia  of  the  brain 
arteries.  Baumgarten  then  declared  that  giant  cells  and 
gummatous  nodules  together  indicated  a  mixed  infection 
of  syphilis  and  tuberculosis.  The  general  opinion  at  the 
present  time  is,  however,  that  true  giant  cells  exist  in  syph- 
ilis without  the  presence  of  tuberculosis. 

Frequently  the  caseation  does  not  lie  in  the  centre  or 
near  the  centre.    The  fatty  degeneration  may  also  be  at  the 


26  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

centre  or  on  the  periphery.  Calcification  does  not  occur  in 
gummata,  which  is  in  sharp  contrast  to  atheromatous 
processes. 

Regressive  Metamorphosis. — In  the  region  regressively 
changed  we  find  either  no  vessels,  or  vessels  almost  entirely 
destroyed.  In  the  periphery  the  numerous  vessels  all 
appear  diseased  with  their  secondary  disturbances  of  nutri- 
tion, which  is  the  cause  of  the  regressive  metamorphosis  of 
the  newly-formed  tissue. 

Besides  the  fatty  and  cheesy  metamorphosis  gummatous 
nodules  may  also  be  changed  into  dense  connective  tissue, 
either  of  part  of  the  nodule,  or  in  its  entirety.  This  takes 

place  especially  under  the  influence 
of  anti  specific  therapy.  We  speak 
then  of  the  cicatrization  of  the  gum- 
matous process.  The  Spirochcete 
pallida  of  Schaudinn  is  only  rarely 
found  in  gummata. 

Gummatous  tumors  begin  their 
growth  in  the  connective  tissue,  both 
in  the  meninges  and  blood-vessels; 


FIG.  11.— Encephalitis  gummosa.    with    the    nerve-tissue    itself    they 

(Personal  observation.)  .  .   .  j_  i  •  i 

have  no  relationship,  except  that  it 
is  secondarily  affected  by  their  presence. 

Secondary  Changes  in  the  Brain  Substance. — By  examina- 
tion with  the  microscope  one  can  see  that  from  the  place 
where  the  gummatous  changes  occur  in  the  meninges  the 
extension  is  directly  into  the  brain  substance.  The  epi- 
cerebral  space  which  normally  exists  between  the  pia  and 
the  cortex  has  disappeared.  The  five  layers  of  Meynert 
on  the  cortex  cannot  be  recognized  any  longer.  The  cells 
either  cannot  be  distinguished  any  more,  or  there  exists  an 
irregular  displacement  of  the  individual  cell  arcades.  The 
blood-vessels  are  surrounded  with  a  cloak  of  round  cells 
which  not  infrequently  conceal  them.  The  cell  proliferation 
penetrates  into  the  external  and  middle  coats  of  the  arteries, 
the  perivascular  lymph  spaces  are  much  dilated  and  in 
part  also  filled  with  round  cells.  One  sees  the  nerve  tissue 
itself  occupied  with  round-cell  nuclei,  and  by  careful  ex- 


PATHOLOGY 


27 


amination  may  observe  numerous  newly  formed  capillaries 
and  capillary  buddings.  The  ganglion  cells  are  changed  in 
many  ways.  Besides  the  above-mentioned  disturbances  of 
their  arrangement  there  is  more  or  less  variation  in  their 
number. 

They  shrink  to  an  insignificant  size,  lose  their  normal 
figure,  their  processes  disappear,  the  nucleus  is  surrounded 
by  only  a  very  thin  covering  of  protoplasm.  In  the  proto- 
plasm one  can  no  longer  recognize  the  Nissl  bodies.  The 
nucleus  resists  destruction  the  longest,  but  that  also  shrinks 
and  finally  becomes  absorbed.  In  the  tissue  one  often  sees 


.'."  '^ 


FIG.  12. — Vessels  in  a  brain  gumma,  surrounded  by  small-celled  infiltration.    Hsematoxylin 
stain.     (Personal  observation.) 

small  and  large  gaps  which  originate  in  part  through  the 
reabsorption  and  disappearance  of  the  ganglion  cells,  and 
in  part  through  the  occurrence  of  an  edema.  The  glia- 
fibres  and  the  star-shaped  connective  tissue  cells  appear 
scattered.  The  medullated  nerve  fibres  stain  poorly  with 
Weigert's  stain  and  appear  irregularly,  swollen,  club- 
shaped,  and  thickened. 

One  sees  in  other  parts  a  transparent,  granular  reticu- 
lated tissue  with  round-celled  nuclei  scattered  through  it 
and  when  this  is  no  longer  present,  then  a  simple  scar  tissue 
presents  itself.  In  such  cases  we  speak  of  an  encephalitis 
syphilitica  where  we  find  in  part  sclerosis  and  atrophy. 


28  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

The  meninges  are  almost  always  involved,  or,  more  cor- 
rectly speaking,  we  have  a  meningo-encephalitis  to  deal  with. 

Syphilitic  Meningitis,  Gummatous  and  Fibrous  Hyperplastic 
Form. — Meningitis  occurs  in  all  of  the  three  coverings  of 
the  brain.  In  the  dura  it  is  connected  sometimes,  etiologi- 
cally  and  locally,  with  a  specific  disease  of  the  bone  or  its 
periosteum.  In  those  cases  in  which  the  meninges  are 
primarily  affected  we  have  the  gumma,  and  the  gumma 
appears  here,  either  as  a  diffuse  infiltration  or  as  a  miliary 
sprinkling  in  the  fibrous  thickenings  and  indurations  of  the 
membranes.  These  gummatous  infiltrations  are  not  to  be 
differentiated  in  their  anatomical  development,  their  sec- 
ondary changes,  or  in  their  behavior  to  the  neighboring 
tissue,  from  the  previously  described  isolated  gummatous 
nodules  and  the  gummatous  infiltration  occurring  in 
encephalitis. 

Meningitis  of  the  gummatous  variety  is  almost  without 
exception  combined  with  a  fibrous  hyperplastic  meningitis. 
The  dura  in  these  cases  is  often  thickened  to  several  times 
its  normal  size.  It  is  usually  adherent  to  the  arachnoid 
and  pia,  with  which  it  presents  an  inseparable  mass.  One 
finds  old  connective  tissue  poor  in  cells  and  newly  formed 
connective  tissue.  Between  the  connective-tissue  fasciculi 
are,  irregularly  in  small  foci  or  as  extensive  infiltration, 
small-celled  proliferations  which  always  follow  the  blood- 
vessels and  spring  from  them.  Where  the  vessels  as  a 
result  of  disease  only  insufficiently  transport  the  blood  or 
through  their  displacement  and  destruction  the  circulation 
is  shut  off,  we  find  caseation  and  necrobiosis. 

Pia  Mater. — In  the  great  majority  of  the  cases  the  lepto- 
meninges  (especially  on  the  base  of  the  brain)  form  the 
starting  point  of  the  disease,  even  where  all  three  meninges 
are  affected.  The  cases  in  which  the  specific  processes  are 
limited  to  the  soft  meninges  are  by  no  means  rare.  In  rare 
cases  the  arachnoid  alone  may  be  affected. 

The  leptomeninges  are  almost  without  exception  adher- 
ent, more  or  less  thickened,  and  with  or  without  gummatous 
deposit,  which  may  appear  as  solitary,  multiple  or  diffuse 
infiltration. 


PATHOLOGY 


29 


»#&':&'# 

W^.'«    •viV*Ji7 


'    '#if~'$"i^&$- 

• '  V4fe^-.  \  'W'  if 


^•••1fe^:-v;'-¥"C;^^;l 
•:  -  ••"*,:  |i  -H¥£S'  vil'y. '-% 


m 


•  '  i  !.:-~..  '.^v-rv^r-v-:-- 
/:•  •  :  .A.  i:-  '•'•  '^'rf^'?^ 
f.-';^.&*tf%!*  '-V* 
'.''.  •  ff'/f^f'^  .^••'•'•;.\ 

-V,  ^j^m^ 

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FIG.  13. — Leptomeningitis  cerebralis  luetica.  a,  inflammatory  infiltration  of  the  soft  mem- 
branes; 6.  the  walls  of  the  pathologically  increased  arteries;  c,  gummata.  Hsematoxylin  stain. 
(Personal  observation.) 


30  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

Under  the  microscope  one  observes  the  destruction  of 
the  normal  configuration  of  the  pia  and  arachnoid,  and  the 
proliferation  of  the  connective  tissue,  changed  by  the  in- 
flammation and  obliterated,  with  only  traces  of  the  elastic 
fibre-rings  recognizable.  Indeed  in  the  leptomeninges  one 
seldom  ever  misses  the  combination  of  the  simple  hyper- 
plastic  type  of  acute  and  chronic  inflammation,  together 
with  the  processes  of  new  tissue  formation,  accordingly 
as  recent  processes  and  later  stages  exist,  which  in  irregu- 
lar sequence  bring  clearly  before  the  eyes  the  chronicity 
and  the  ever-recurring  relapses  characteristic  of  a  syph- 
ilitic process.  The  less  the  inflammatory  congestive  element 
prevails,  the  greater  appears  the  evidence  of  the  specific 
neoplastic  type  of  inflammation. 

Simple  Meningitis. — With  regard  to  the  occurrence  of  a 
simple  meningitis  in  syphilis  Virchow  has  expressed  a 
doubt,  and  has  emphasized  the  point  that  in  cases  of  firmly 
established  meningitis  simplex  the  objection  may  be  raised 
that  previously  a  gummatous  process  existed  which  had 
receded.  Heubner  and  later  Buttersack  also  hold  this  opin- 
ion. Schultze  had  previously  declared  it  possible  that  gum- 
inatous  meningitis  had  begun  the  specific  process  and  then 
receded,  leaving  only  a  simple  thickening  of  the  meninges, 
which  later  at  the  pathological  examination  presented  noth- 
ing specific  in  character. 

Oppenheim  is  opposed  to  this  view  and  is  inclined  to 
regard  meningitis  simplex  as  a  not  uncommon  result  of 
syphilis. 

Localization  of  the  Meningitis. — The  syphilitic  meningitis 
is  either  distributed  uniformly  on  the  base  and  convexity  of 
the  brain  or  (this  is  seldom)  it  is  localized.  Oppenheim 
reports  a  case  in  which  the  entire  convexity  over  the  frontal 
and  parietal  lobes  was  affected.  A  case  of  very  extensive 
leptomeningitis  chronica  is  portrayed  by  Bechterew  (Fig. 
14). 

Usually  the  above-described  form  of  meningitis  is  local- 
ized on  the  base  of  the  brain.  In  few  cases  of  syphilis  of 
the  nervous  system  does  one  find  the  brain  base  free  from 
pachy  and  leptomeningitis. 


PATHOLOGY  31 

The  favorite  location  on  the  base  is  the  region  of  the 
chiasm  and  interpeduncular  space.  The  nerves  of  the  eye 
muscles  and  the  optic  nerve  are  particularly  often  affected 
by  the  specific  exudate.  The  meningeal  affection  extends 
also  very  frequently  to  the  region  of  the  arteria  fossae  sylvii. 

In  eighteen  autopsies  in  cases  of  brain  syphilis,  from 
1892  to  1901,  I  found  meningitis  of  the  base  twelve  times. 

The  basilar  exudate  in  specific  meningitis  resembles  the 
tubercular  variety  in  that  it  usually  involves  the  whole  base. 
There  are,  however,  exceptions  to  this.  Oppenheim  states 
that  sometimes  only  the  optic  chiasm  or  a  part  of  the  motor 
oculi  nerve  is  affected.  He  reports  one  case  in  which  the 


FIG.  14. — Leptomeningitis  chronica  with  general  cloudiness  of  the  soft  meninges.    (Bechterew.) 

gummatous  infiltration  involved  only  a  small  portion  of 
the  middle  part  of  the  optic  chiasm.  In  other  cases  reported 
by  him  the  specific  growth  involved  the  trigeminus,  the 
ganglion  gasseri,  or  the  individual  nuclei  of  other  cranial 
nerves.  I  have  never  had  the  opportunity  of  seeing  such  a 
case,  but  one  should  not  forget  that  an  isolated  specific 
meningitis  may  injure  the  individual  cranial  nerves. 

Injury  to  the  Structures  on  the  Base. — Let  us  consider  the 
injury  done  to  the  nervous  formation  by  syphilitic  basilar 
meningitis.  The  brain-tissue  on  the  base  is  affected  natur- 
ally much  in  the  same  way  as  the  changes  described  as 
occurring  on  the  brain  convexity,  but,  in  addition,  we  must 


32 


SYPHILIS  AND  THE  NERVOUS  SYSTEM 


consider  here  the  damage  which  takes  place  in  the  large 
arteries  and  the  cranial  nerves.  The  vessels  may  become 
involved  through  an  extension  of  the  specific  gummatous 
or  inflammatory  processes  to  their  walls  or  through  simple 
compression. 

The  nerve-trunks  suffer  in  various  ways.     The  luetic 
process   may  encroach   on  them   and   then  independently 


FIG.  loa. — Leptomeningitis  basalis  chronica.     (Personal  observation.) 

proliferate  into  the  nerve-fibres  or  by  mechanical  compres- 
sion or  scar  formation  injure  them.  The  combination  of 
disease  of  the  membranes  together  with  analogous  changes 
in  the  nerves  themselves  is  well  recognized.  Dittrich  de- 
scribes a  case  of  specific  amaurosis  together  with  cranial 
caries  of  the  frontal  bone  and  a  thickening  of  the  dura 
mater  with  compression  and  specific  disease  of  the  optic 
nerve. 


PATHOLOGY 


33 


We  will  discuss  later  the  question  of  whether  the  nerves 
are  primarily  affected  by  syphilis  or  not ;  also  the  question 
as  to  whether  a  specific  process  existing  in  the  arteries 
which  supply  the  cranial  nerves  may  involve  these  nerves 
directly  without  the  instrumentality  of  the  meninges. 

The  optic  nerves  and  the  nerves  supplying  the  eye 
muscles  are  most  frequently  involved  in  a  specific  basilar 
meningitis,  relatively  frequently  the  facial  is  affected, 
more  seldom  the  trigeminus  and  still  more  seldom  the  last 
four  cranial  nerves,  the  glosso-pharyngeal,  vagus,  accessory 
and  hypoglossal. 


N.  III. 


FIG.  156.  —  Meningitis  basalis  syphilitica.  Transverse  section  from  the  region  of  the  corpora 
mammillaria  and  the  oculomotor  nerve.  Magnifying  glass  enlargement  (Oppenheim).  N.III, 
oculomotor  nerve;  Sy.,  syphilitic  growth;  C.M.,  corpora  mammillaria. 

Disease  of  the  Blood-vessels  and  Arteries.  —  Disease  of  the 
blood-vessels  of  the  nervous  system  is  a  most  important 
subject.  The  vessels  may  be  mechanically  compressed 
through  meningeal  thickening  or  gummatous  tumors,  which 
leads  on  the  one  hand  to  displacement  of  the  lumina  of  the 
vessels  or  on  the  other  to  thrombus  formation.  .Steenberg 
was  one  of  the  first  to  demonstrate  that  the  arteries  in 
themselves,  in  brain  syphilis,  frequently  may  be  the  seat  of 
pathological  changes.  He  called  attention  to  the  frequent 
thickening  of  the  walls  of  the  brain  arteries,  the  narrowing 
of  their  lumina  and  likewise  their  displacement.  A  further 
advance  was  made  when  it  was  recognized  that  the  vessels 
also  could  be  the  starting  point  of  specific  gummatous 


34 


SYPHILIS  AND  THE  NERVOUS  SYSTEM 


growths.  Heubner  was  the  first  observer  to  direct  universal 
attention  to  the  study  of  specific  disease  of  the  blood-vessels. 
Heubner  taught  that  the  infectious  agent  of  syphilis 
produced  primarily  an  irritation  of  the  endothelium  of  the 
blood-vessels  of  the  brain,  and  that  the  proliferation  of  the 
endothelium  was  the  beginning  of  the  entire  process.  Only 
later  did  this  irritation  extend  to  the  vasa  nutricia  and 
then  to  a  genuine  inflammatory  process  of  the  adventitial 
connective  tissue.  The  proliferation  of  the  intima  may 
in  the  further  development  of  the  process  remain  stationary 
as  such  or  be  transformed  into  ordinary  connective  tissue. 
Caseation,  fatty  degeneration,  and  calcification  do  not  take 
place.  The  process  of  the  proliferation  of  the  intima  leads 

naturally  to  a  narrowing,  and 
in  the  end  obliteration  of  the 
vessel  lumen.  Heubner  empha- 
sized the  fact  that  this  process 
may  occur  in  young  people.  He 
distinguishes  this  process,  which 
he  calls  arteriosyphilis,  from 
arteriosclerosis  anatomically  by 
the  absence  of  regressive  meta- 
morphosis, the  greater  tendency 
of  the  process  to  be  circum- 
scribed in  character,  while  in 
arteriosclerosis  it  is  more  general,  and  finally  to  the  pre- 
ponderating preference  of  the  latter  for  development  in 
the  large  arteries,  whereas  in  arteriosyphilis  the  medium- 
sized  arteries  are  the  most  often  affected. 

The  position  represented  by  Steenberg  and  Lancer eaux 
that  the  majority  of  the  clinical  symptoms  of  syphilis  are 
caused  by  changes  in  the  arteries  and  their  secondary  conse- 
quences is  now  accepted  as  final. 

Heubner  declared  that  syphilitic  arterial  disease  was  of 
a  characteristic  type.  The  process  above  described  as  pre- 
sented by  him  was  the  exact  antithesis  of  the  teaching  of 
Virchow  and  Wagner,  which  up  to  this  time  had  been  the 
accepted  view.  The  latter  regarded  the  rapid  disintegra- 
tion of  the  cells  as  the  chief  factor  in  the  specific  process, 


FIG.  16. — Heubner's  arterial  disease. 
Infiltration  of  the  adventitia,  media,  in- 
tima. Marked  proliferation  of  the  in- 
tima a,  adventitia;  b,  media;  c,  intima. 
(Personal  observation.) 


PATHOLOGY 


35 


while  the  former  considered  increase  in  cell  activity  and 
proliferation  the  characteristic  element. 

In  1872  Cornil  and  Ranvier  demonstrated  in  wounds, 


FIG.  17. — Section  of  a  gumma  from  the  brain-base.  Hsematoxylin  stain.  In  all  the  vessels 
the  lumina  are  much  narrowed,  at  figure  a,  obliterated;  in  the  lumina  of  the  other  vessels  is 
some  blood  b  (red  blood-corpuscles).  The  narrowing  of  the  lumen  is  due  to  the  proliferation 
of  the  intima  c.  Media  and  adventitia  are  hidden  by  inflammatory  infiltrated  tissue.  (Personal 
observation.) 

ulcers,  and  other  inflammations,  that  a  narrowing  and  oblit- 
eration of  the  blood-vessels  occurred  which  was  similar 
in  character  to  the  obliterating  vessel  inflammation  which 


36  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

sets  in  after  the  '  application  of  a  ligature,  and  that  the 
endarteritis  syphilitica  of  Heubner  had  nothing  of  a  specific 
nature  in  itself,  although  these  observers  did  not  deny  a 
special  predisposition  on  the  part  of  luetic  individuals  to 
arteritis,  and  particularly  of  the  brain  arteries. 

According  to  Koster  the  thickening  of  the  intima  is 
closely  related  and  dependent  upon  areas  in  the  media 
which  have  developed  from  the  vasa  vasorum.  Endarteritis 
occurs  only  in  arteries  which  are  supplied  with  the  vasa 
nutricia.  For  this  reason  endarteritis  is  so  often  found  in 
the  brain,  because  there  even  very  small  arteries  are  pro- 

vided   with    the     vasa 
vasorum,  and  also  for 

I  *  the   same  reason  arte- 

k  *.  //('      «  ^  %  ries  of  a  similar  calibre 

*?  '  '  '  \    '     .4 

in   the  extremities   are 


»*  spared.     The  generally 

•    .^  accepted  opinion  at  the 

'"";      s,\.  present  time  is  the  one 

*  »  of  Cornil  and  Eanvier 

c,  that  the  endarteritis  of 

Heubner  has  nothing  in 


FIG.  18.  —  The  remnants  of  a  vein  (phlebosclero-       itself    which    is 
sis)  .     One  sees  in  the  necrotic  tissue  still  some  elastic 
fibres,  arranged  in  the  form  of  rings.    Stain  Weigert's       teristic  of  lU6S,  that  tllC 
elastica  method.     (Personal  observation.)  .  . 

proliferation  of  the  in- 

tima is  secondary  in  nature,  and  that  the  inflammatory  proc- 
ess proceeds  from  the  vasa  vasorum.  Strasmann  and 
Beitzke  in  a  recent  study  of  this  subject  have  also  concluded 
that  in  the  vasa  vasorum  and  the  perivascular  lymph-spaces 
the  starting  point  of  syphilitic  vessel  disease  is  to  be  found. 

Ernst  Meyer  in  1897,  after  a  comprehensive  study  of 
this  subject,  came  to  the  conclusion  that  the  endarteritis 
of  the  brain  arteries,  in  so  far  only  as  it  appears  inde- 
pendently and  involves  a  wide  distribution,  can  with  a 
certain  definiteness  be  regarded  as  specific. 

So  far  as  I  know  the  Spirochcete  pallida  has  been  demon- 
strated only  once  in  a  case  of  endarteritis  syphilitica  cere- 
bralis.  This  was  in  a  case  of  Benda's  where  the  specific 


PATHOLOGY  37 

nature  of  the  changes  could  be  proven  by  the  small  area 
of  necrosis  within  the  cell  infiltrate. 

The  Veins. — There  is  also  a  voluminous  literature  con- 
cerning the  disease  of  the  veins  in  syphilis.  Charcot  de- 
scribes the  veins  enveloped  as  in  a  muff  by  nuclei.  There 
is  a  uniform  gradually  increasing  thickening  and  cell  infil- 
tration of  the  vein  walls,  finally  terminating  in  an  oblitera- 
tion of  the  lumen  of  the  vein. 

"We  recognize  in  chronic  disease  of  the  central  nervous 


FIG.  19. — Vein  from  a  section  from  menmguis  encephalitis  luetica  (gummosa).  Weigert's 
elastica  stain.  The  lumen  of  the  vein  is  obliterated  by  granulation  tissue;  the  intima  and  media 
are  destroyed  and  supplanted  by  recent  connective  tissue;  in  place  of  the  adventitia  is  only 
inflammatory  tissue.  Nothing  remains  to  indicate  a  former  vein  except  the  thickened  and 
broken-up  elastica  interna  and  externa.  (Personal  observation.) 

system  as  different  types  of  vein  affections  (1)  perivascular 
infiltrations  of  granulated  tissue;  (2)  peri-  and  endophle- 
bitis, or  simply  phlebitis,  a  disease  of  all  parts  of  the  walls 
of  the  vein;  (3)  the  phlebosclerosis  of  Eieder;  (4)  a  true 
endophlebitis  consisting  of  a  large-celled  proliferation  of 
the  intima  or  as  a  fibrous  and  hyaline  thickening  of  the 
intima.  A  very  interesting  form  of  endophlebitis  prolifer- 
ans  obliterans  or  tuberosa  described  by  Bartels  perhaps 
also  belongs  here.  Although  in  the  history  of  the  case  evi- 
dence of  syphilis  was  not  obtainable,  and  although  neither 
clinically  nor  at  the  autopsy  were  signs  of  syphilis  discov- 


38 


SYPHILIS  AND  THE  NERVOUS  SYSTEM 


ered,  Bartels,  by  exclusion  and  upon  the  fact  of  an  absolute 
absence  of  involvement  of  the  arteries,  remembering  that 
Bieder  had  demonstrated  in  syphilis  that  the  venous  system 
can  be  affected  long  before  the  arterial,  came  to  the  conclu- 
sion that  syphilis  could  be  assumed  as  the  etiological  factor. 
Arteriosclerosis  in  Syphilitics. — It  is  important  to  further 
discuss  the  question  of  arteriosclerosis  and  syphilis.  It  is 
a  well-known  fact  that  very  often  following  a  syphilitic  in- 
fection arteriosclerosis  is  found  in  young  persons,  much 
younger  than  the  age  of  forty-five,  which  one  considers  as 
about  the  time  of  life  for  the  beginning  in  the  arterial 


FIG.  20. — Numerous  elastic  rings  in  arteries.    Stain  Weigert's  elastica  method.     (Personal 

observation.) 

system  of  arteriosclerotic  changes.  In  order  to  bring  this, 
arteriosclerosis  into  a  relationship  with  a  past  syphilis, 
naturally  it  is  important  to  exclude  other  causative  factors, 
such  as  chronic  alcoholism,  abuses  tabaci,  severe  joint  rheu- 
matism, and  physical  overexertion.  The  presence  of  a 
severe  arteriosclerosis  in  a  comparatively  young  person, 
if  we  are  able  to  exclude  the  above-mentioned  factors,  justi- 
fies us  first  of  all  in  thinking  of  syphilis.  One  should  of 
course  not  overlook  the  fact  that  from  time  to  time  cases 
are  observed  where  one's  entire  etiological  experience  fails 
him.  Such  cases  occur  usually  where  a  family  predisposi- 
tion to  arteriosclerosis  exists.  It  is  to  be  remembered  also 
that  neither  the  localization  nor  the  extent  of  the  atheroma- 


PATHOLOGY 


39 


tosis  can  be  utilized  with  certainty  for  determining  a  specific 
origin.  The  ordinary  arteriosclerosis  may  be  general  in 
character,  or  localized  to  a  particular  region,  and  may  stop 
short  in  the  large  vessels  as  well  as  in  the  medium-  and 
small-sized  arteries.  The  endarteritis  of  Heubner  is  like- 
wise often  limited  to  small  areas. 

I  possess  preparations  from  two  cases  which  demon- 
strate this  most  clearly.    In  these  cases  of  two  young  men, 


:;•••/•$•/•::-•'•••• 
*./,••//•  '• 


FIG.  21. — Obliterated  arteries  in  a  case  of  specific  basilar  meningitis.  The  split  elastica  is 
alone  preserved.  (Personal  observation.)  Staining  by  Weigert's  elastic  method.  (Personal 
observation.) 

twenty-four  and  twenty-eight  years  of  age  respectively,  the 
endarteritis  was  localized  in  the  beginning  portion  of  the 
arteria  fossae  sylvii,  both  cases  had  a  specific  hemiplegia, 
and  both  died  as  the  result  of  a  severe  intercurrent  pneu- 
monia which  gave  the  opportunity  for  the  postmortem. 
The  other  brain  arteries  as  well  as  the  large  arteries  of 
the  body  were  macroscopically  normal. 


40 


SYPHILIS  AND  THE  NERVOUS  SYSTEM 


The  Syphilitic  Aortitis  of  Heller-Dohle. — There  has  been 
much  discussion  recently  as  to  whether  there  is  a  clear-cut 
difference  between  an  atheromatosis  of  specific  origin  and 
one  of  the  usual  type.  Heller  and  Dohle  have  described  a 
somewhat  typical  picture  of  a  specific  aortic  sclerosis. 
Marchand  designates  the  specific  aortic  changes  as  "  in- 
durated sclerosis."  Heller,  Marchand  and  Heiberg  have 
described  the  distinguishing  specific  features  as  consisting 


FIG.  22. — Small  area  of  softening  in  the  internal  capsule  caused  by  the  specific  shutting 
off  of  the  arteria  fossa  sylvii.     (Personal  observation.) 

of  rugas  with  fossag  lying  between  them.  All  observers 
agree,  however,  that  the  seat  of  the  syphilitic  aortitis^  lies 
in  the  beginning  part  of  the  ascending  aorta,  while  on  the 
other  hand  the  common  aortitis  is  chiefly  situated  in  the 
descending  aorta. 

Microscopically  specific  aortitis  is  differentiated  from 
the  non-specific  through  the  primary  seat  of  the  changes  in 
the  adventitia  and  media,  while  in  simple  aortitis  their  be- 
ginning and  preponderance  occur  in  the  intima.  Reuter, 
also  Schmorl  at  Dresden  and  Wright  at  Boston,  have 
demonstrated  the  Spiroch&te  pallida  in  aortas,  which 
showed  evidences  of  the  Dohle-Heller  type  of  aortitis.  The 
spirochaete  was  found  in  the  endothelial  proliferations  of 
these  aortas.  These  cases  prove  at  any  rate  that  this  variety 
of  aortitis  can  be  of  specific  origin.  E.  Fraenkel  was  the 


PATHOLOGY 


41 


first  to  obtain  the  Wassermann  reaction  in  a  case  of  aortitis 
as  described  by  Dohle  and  Heller.  Since  then  this  finding 
has  been  almost  a  constant  one. 

Aneurism. — Much  more  seldom  than  is  the  case  in  the 
arteriosclerosis  of  age,  aneurism  occurs  through  specific 
disease  of  the  brain  arteries.  It  is  most  often  to  be  found 
in  the  arteria  fossae  sylvii  and  the  arteria  basilaris. 


Fia.  23. — Localized  arteritis  of  Heubner.  A  little  toward  the  left  from  the  middle  of  the 
base  of  the  pons  one  sees  the  arteria  basilaris,  which  for  a  short  distance  is  severely  affected 
(Personal  observation.) 

Summary  of  the  Vessel  Disease  in  Syphilis. — If  we  review 
the  various  opinions  concerning  specific  disease  of  the  blood- 
vessels the  following  conclusions  will  become  evident: 

First.  The  blood-vessels  can  become  affected  by  syphilis 
in  a  purely  mechanical  manner  both  in  the  meninges  and 
the  nerve-tissue  itself. 

Second.  They  become  involved  also  by  the  specific 
process  in  the  vicinity  per  contiguitatem,  that  is,  the  gum- 


42  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

matous  or  inflammatory  process  attacks  the  arterial  walls 
by  extension  and  may  present  a  peri-,  meso-,  and  pan- 
arteritis,  either  with  or  without  true  gummatous  symptoms. 

Third.  The  vessels  may  become  diseased  in  another 
way.  An  inflammation  of  the  capillaries  produced  by  the 
toxin  of  syphilis  leads  to  an  inflammatory  affection  of  the 
walls  involving  primarily  the  media  and  adventitia,  second- 
arily the  intima  becomes  involved,  and  it  may  then  inde- 
pendently further  proliferate.  This  is  the  picture  of  the 
endarteritis  of  Heubner  with  narrowing  and  obliteration  of 
the  lumen  and  is  the  form  most  commonly  found.  This 
variety  of  arteritis  may  be  accompanied  by  a  gummatous 
infiltration. 

Fourth.  Syphilis  tends  to  produce  an  atheromatous 
degeneration  of  the  arteries,  one  form  of  which  degenera- 
tion cannot  be  differentiated  from  the  ordinary  atheroma- 
tous changes;  the  other,  the  arteritis  of  Dohle  and  Heller, 
should  be  regarded  as  extremely  suspicious  of  syphilis. 

The  Results  of  Disease  of  the  Blood-vessels  in  Cerebral 
Lues. — The  above-described  changes  in  the  vessels  have 
collectively  as  their  final  result  the  narrowing  of  the  vessel 
lumen  and  frequently  the  complete  displacement  of  the 
same.  Whether  through  thrombosis  or  obliteration,  the  cir- 
culation is  shut  off,  and  as  a  consequence  of  this,  severe 
disturbances  of  nutrition,  sometimes  amounting  to  necrotic 
softening  of  the  territory  belonging  to  the  vessels,  are  pro- 
duced. Although  the  arteries  on  the  convexity  as  well  as  on 
the  base  of  the  brain  may  become  diseased,  yet  the  softening 
in  the  cortex  and  the  medullary  layer  underneath,  likewise 
on  the  base  of  the  brain  and  the  tissue  lying  immediately 
above  it,  occurs  much  less  often  than  in  the  interior.  The 
large  ganglia,  the  internal  capsule,  the  pons  and  medulla 
may  be  regarded  as  the  areas  of  the  brain  which  seem  to 
have  a  predisposition  to  softening.  In  the  cortex  there 
exists  to  a  large  degree  the  possibility  of  the  occur- 
rence of  a  collateral  circulation.  The  areas  of  softening 
which  are  produced  by  the  shutting  off  the  vessel  lumen 
caused  by  specific  disease  cannot  be  differentiated  from  the 
softening  due  to  the  usual  lumen  obliteration.  "We  find  here 


PATHOLOGY  43 

also,  through  the  reabsorption  of  the  broken-down  and 
necrotic  tissue  and  through  the  productive  inflammation  of 
the  neurologia,  secondary  sclerosis  and  thickening  of  the 
affected  nervous  tissue. 

Genuine  inflammatory  processes  in  the  neighborhood  of 
ischsemic  softenings  do  not  occur  as  a  consequence  of  specific 
disease  of  the  arteries,  but  the  infiltration  of  the  vessel  walls 
and  proliferation  of  the  neurologia  cells  may  be  regarded 
solely  as  reactive  in  character. 

Rupture  and  Secondary  Hemorrhages. — A  further  result 
of  disease  of  the  arteries  is  rupture  and  secondary  hemor- 


Fio.  24. — Necrosis  of  the  right  frontal  lobe  on  the  base  in  a  case  of  endarteritis  luetica.  (Schaffer.) 

rhages.  Ruptures  occur  with  and  without  aneurism  forma- 
tion. Where  the  elastic  tissue  is  much  diminished  or  en- 
tirely destroyed,  where  the  muscularis  of  the  media  is 
supplanted  by  less  resistant  connective  tissue,  the  latter 
without  any  pouching  of  the  intima  may  give  way  to  the 
pressure  of  the  blood  and  make  hemorrhage  possible. 

It  is  well  to  remember  that  in  the  examination  of  prep- 
arations containing  specifically  diseased  vessels  one  seldom 
encounters  aneurism  formation,  also  that  the  often-occur- 
ring thickening  of  the  adventitia  and  of  the  tissue  adjoining 


44  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

the  vessels  is  somewhat  of  a  protection  against  hemorrhage. 
Hemorrhages  in  the  interior  of  the  brain  are  not  frequent, 
because  the  vessels  there  are  not  so  often  affected,  never- 
theless a  vast  number  of  hemorrhages  in  this  region  have 
been  observed.  Gowers  has  observed  relatively  frequent 
conditions  of  sclerosis  and  induration.  He  believes  in  such 
cases  the  vessel  changes  do  not  permit  a  normal  nourish- 
ment of  the  diseased  portions,  but  that  the  circulation  has 
not  been  sufficiently  impeded  to  produce  an  actual  necrosis. 

Combination  of  Different  Pathological  Forms. — From  a  re- 
view of  the  literature  pertaining  to  this  subject  it  is  evident 
that  one  finds  in  syphilis  very  often  a  combination  of  the 
pathological  changes  which  we  have  been  considering.  This 
variety  of  anatomical  change,  although  not  pathognomonic 
of  syphilis,  is  an  important  criterion  in  the  diagnosis. 

Differential  Diagnosis  in  Specific  Processes. — The  question 
has  often  been  debated,  whether  out  of  the  pathological 
changes  alone,  without  any  further  information,  the  specific 
nature  of  the  disease  can  be  determined.  A  pathology  which 
is  absolutely  pathognomonic  of  syphilis  does  not  exist. 

Sarcoma. — There  are  cases  of  diffuse  sarcomatous 
growth  of  the  meninges  of  the  brain  and  spinal  cord  which 
bear  some  resemblance  to  the  diffuse  gummatous  infiltration 
found  in  syphilis.  There  are  marks  of  differentiation,  how- 
ever. We  do  not  find  in  these  cases  the  almost  never-failing 
signs  of  a  regressive  metamorphosis  found  in  syphilis.  The 
spinal  cord  is  only  seldom  involved,  when  we  exclude  the 
injury  done  through  compression,  while  in  syphilis  of  the 
brain  and  spinal  cord  we  have  a  meningo-encephalitis  or 
meningomyelitis. 

An  endarteritis  occurs  in  new  growths  as  well  as  in 
specific  conditions,  but  in  the  former  is  not  found  developed 
to  such  a  great  extent  nor  high  degree  as  in  the  latter.  The 
marked  infiltration  of  the  external  membranes  and  the 
sheaths  of  the  vessels,  as  well  as  the  inflammatory  infiltrate 
of  the  surrounding  tissue,  is  also  lacking.  A  differentiation 
between  these  two  pathological  entities  is  not  very  difficult. 

Cysticercus. — The  same  is  true  of  the  cysticercus-menin- 
gitis.  We  find  here  also  chronic  basilar  arachnoiditis  and 


PATHOLOGY 


45 


leptomeningitis  together  with  and  without  a  secondary 
hydrocephalus  and  an  obliterating  arteritis.  In  the  major- 
ity of  cases  the  diagnosis  can  be  determined  macroscopi- 
cally  by  the  presence  of  cysticercus  vesicles.  Stertz  reports 
a  case  in  which  he  was  able  to  demonstrate  the  cysticercus 
vesicles  in  the  spinal  fluid  by  means  of  lumbar  puncture 
during  life. 

Tuberculosis. — In  tuberculosis  the  condition  is  different. 
The  gummata  may  attain  the  same  size  as  the  tubercles. 
They  often  appear  as  miliary  in  character.  The  miliary 
gummata  have  a  predilection  for  the  base  of  the  brain  and 


FIG.  25. — Disease  of  an  artery  in  tubercular  leptomeningitis.  Weigert's  elastic  stain. 
(Personal  observation.)  a,  the  intima  has  proliferated  and  is  infiltrated;  b,  media;  c,  adventitia. 
The  artery  is  closely  imbedded  in  inflammatory  tissue.  In  the  arterioles  (d)  the  same  process 
is  shown.  The  surrounding  tissue  (e)  is  in  part  necrotic. 

the  course  of  the  large  vessels,  and  they  are  more  often 
found  distributed  in  the  meninges  than  in  the  nerve-tissue 
itself.  The  caseation  in  both  tuberculosis  and  syphilitic 
nodules  may  be  the  same.  The  inflammatory  structure  of 
the  surrounding  tissue  may  in  both  forms  of  growth  be  pres- 
ent or  absent.  The  microscopical  finding  does  not  present 
any  means  of  distinguishing  the  nature  of  the  affection,  for 
in  gummata  giant  and  epithelioid  cells  are  also  found  and, 
as  is  well  known,  tubercular  bacilli  cannot  always  be  demon- 
strated in  tubercular  lesions. 


46 


SYPHILIS  AND  THE  NERVOUS  SYSTEM 


Comparing  the  two  forms  of  meningitis,  chronic  tuber- 
cular as  well  as  the  syphilitic  type  can  be  diffuse  or  circum- 
scribed in  character.  The  involvement  of  the  brain  sub- 
stance can  in  both  processes  be  an  extensive  one  and  in  both 
an  acute,  subacute,  or  chronic  type  may  exist.  In  syphilis, 
as  well  as  tuberculosis,  the  process  develops  from  the  pia 
septum  and  the  vessels. 

The  involvement  of  the  arteries  in  tuberculosis  may  also 
be  extensive  and  severe. 


* 

ISi 


FIG.  26. 

A  glance  at  Fig.  26,  which  is  taken  from  a  case  of  tuber- 
cular meningo-encephalitis,  will  show  the  marked  prolifera- 
tion of  the  intima,  which  has  brought  about  a  high  degree  of 
narrowing.  One  will  also  observe  here  the  inflammatory 
infiltration  in  the  neighborhood  of  the  arteries.  Hoche,  in 
a  classical  case  of  tuberculosis  of  the  spinal  cord,  found  and 
described  a  phlebitis  obliterans.  Small  tubercles  micro- 
scopically as  well  as  gummata  may  be  observed  in  the  walls 
of  the  arteries.  Also  the  outgoing  cranial  nerves  and  spinal 


PATHOLOGY  47 

cord  roots  may  be  either  compressed  or  infected  by  the 
meningeal  exudate  as  in  a  syphilitic  process. 

For  these  reasons  Rumpf  has  stated  that  only  in  the 
absence  of  all  other  infections,  or  where  specific  or  tuber- 
cular findings  are  present  in  other  organs  of  the  body 
(tubercle  bacilli  not  being  present),  can  the  pathological 
diagnosis  be  determined  beyond  a  doubt. 

The  following  case  will  illustrate  the  difficulties  in  a 
differential  diagnosis: 

A  laborer  as  the  result  of  severe  epileptic  convulsions 
died  in  the  hospital.  The  provisional  diagnosis  of  idio- 
pathic  epilepsy  was  made.  The  postmortem  showed  entirely 
normal  internal  organs.  There  was  no  evidence  of  tuber- 
culosis or  syphilis,  past  or  present.  On  the  inner  side  of 
the  dura  over  the  upper  middle  portion  of  the  left  parietal 
region  there  was  a  tumorous  formation  which  reached,  in 
many  places,  into  the  brain  cortex  and  pushed  itself  wedge- 
like  in  between  the  convolutions.  The  rest  of  the  brain  was 
normal. 

The  prosector  and  a  surgeon  present,  with  equal  patho- 
logical experience  to  the  prosector,  were  of  diverse  opinion. 
The  one  assumed  the  nature  of  the  lesion  as  tubercular,  the 
other  as  gummatous.  The  microscopical  examination  pre- 
sented the  picture  of  foci  of  caseation  with  no  giant  cells 
and  no  tubercular  bacilli.  On  the  other  hand  the  vessel 
changes  were  so  numerous  and  of  so>  marked  a  character  in 
the  form  of  obliteration  of  both  arteries  and  veins,  together 
with  so  classical  a  type  of  Eieder's  phlebosclerosis,  that  the 
gummatous  nature  of  the  lesion  could  not  be  doubted. 

The  general  opinion  in  regard  to  the  pathological  diag- 
nosis of  syphilis  at  the  present  time  may  be  summed  up  in 
the  following  manner.  The  changes  found  in  the  manifold 
lesions  of  lues  present  nothing  which  may  not  be  demon- 
strated in  simple  inflammatory  lesions,  but  the  specific 
lesions  in  their  life  history,  their  localization  and  occur- 
rence, and  finally  in  their  combinations,  present  character- 
istics which  furnish  tangible  proofs. 

Primary  Disease  of  the  Nervous  System. — Thus  far  we  have 
seen  that  the  nervous  tissue  itself  has  remained  primarily 


48  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

intact,  and  has  only  been  involved  in  secondary  changes.  It 
has  atrophied  because  pressed  upon  by  gummatous  pro- 
liferations and  meningeal  indurations;  it  has  become 
necrotic  because  of  lumen-obliteration  and  deficient  blood- 
supply. 

Gilbert  and  Lion  have  described  a  form  of  inflammation 
in  the  spinal  cord  which  does  not  bear  the  character  of  the 
specific  granulation  tissue  and  the  specific  disease  of  the 
blood-vessels  and  have  designated  this  form  as  "meningo- 
myelite  diffuse  embryonnaire. "  They  have  stated  in  this 
type  that  softening  of  the  nervous  tissue  may  be  produced 
by  the  direct  influence  of  syphilis. 

Oppenheim  likewise  declares  that  syphilis  can  cause  a 
simple  encephalitis.  Pie  bases  his  opinion  upon  an  old  case 
reported  by  Charcot  and  Gombault.  In  this  case  there  were 
numerous  lesions  in  the  left  cerebral  peduncle,  on  the  under 
surface  of  the  pons?  and  the  region  of  the  corpora  quadri- 
gemina.  The  lesions  were  recognizable  macro scopically, 
and  microscopically  were  shown  to  consist  of  a  peripheral 
layer  of  neurologia  cells  and  a  neutral  zone  of  fatty  granular 
cells.  In  1905  Barrett  and  Danvers,  in  Massachusetts, 
described  a  case  of  brain  syphilis  where  there  existed  side 
by  side  gummatous  changes  in  the  brain  tissue,  and  simple 
and  gummatous  meningitis,  Heubner's  arteritis  with  the 
secondary  processes  of  softening  and  a  multiple  dissem- 
inated encephalitis,  which  was  independent  of  the  disease 
in  the  meninges  and  was  able  to  be  distinguished  by  the 
genuine  inflammatory  symptoms  around  the  blood-vessels, 
the  appearance  of  recent  granulation  tissue,  degenerative 
changes  in  the  nerve  cells,  and  a  secondary  reactive  prolif- 
eration of  the  glia.  Barrett  called  this  process  "dissem- 
inated syphilitic  encephalitis." 

Numerous  observations  of  Oppenheim,  Siemerling,  and 
Kostentisch  indicate  that  the  nerve  nuclei  are  also  subject 
to  primary  degeneration  through  the  influence  of  syphilis. 
The  cerebral  nerves  may  likewise  present  a  simple  atrophy. 

For  statistics  in  regard  to  the  frequency  of  occurrence 
of  the  individual  forms  of  brain  syphilis  we  are  indebted 
to  the  syphilitic  clinic  in  Vienna.  In  29  cases  of  brain 


PATHOLOGY  49 

syphilis  there  occurred  arteritis  of  the  arteria  fossae  sylvii 
14  times ;  closure  of  the  right  internal  carotid,  right  arteria 
foss£e  sylvii  and  foramen  of  Magendie  of  each  one ;  two  cases 
of  cerebral  atrophy,  hydrocephalus  internus  two  times, 
encephalomalacia  eleven  times,  thickening  and  adhesion  of 
the  dura  with  the  cranium  one  time,  coincident  disease  of 
the  skull  two  times,  gummatous  processes  and  circumscribed 
gummata  eight  times,  encephalitis  and  leptomeningitis  three 
times,  and  periencephalitis  once. 

In  a  review  of  185  cases  of  syphilis  of  the  nervous  system 
I  found  128  cases  of  cerebral  and  29  of  spinal  lues.  From 
these  cases  the  arterial  form  appeared  43  times,  the  menin- 
geal  128,  and  in  14  cases  there  was  a  combination  of  both 
forms.  In  a  review  made  by  Patrick  in  1901  specific  arter- 
itis appeared  as  the  most  frequent  type,  and  next  in  fre- 
quency came  the  syphilitic  meningitis. 

We  may  accept  at  the  present  time,  without  the  influence 
of  the  specific  syphilitic  changes  in  the  vessel  and  connec- 
tive-tissue apparatus  of  the  nervous  system,  in  rare  in- 
stances that  there  exists  a  primary  degeneration  of  the 
nerve  tissue  itself  in  the  form  of  an  encephalitis,  a  soften- 
ing, an  induration  and  sclerosis,  and  a  simple  atrophy  of 
nerve  nuclei. 


in 

ETIOLOGY  OF  NERVOUS  LUES  AND  SPECIFIC 
ENDARTEBITIS 

BEFOEE  we  discuss  the  symptomatology  of  brain  syphilis, 
it  is  of  importance  to  consider  the  etiology  of  nervous 
syphilis.  Since  the  statistical  presentations  on  this  subject 
by  Rumpf  and  Naunyn  we  know  that  not  infrequently  within 
the  first  year  of  infection,  sometimes  indeed  within  the  first 
few  months,  syphilis  can  cause  organic  disease  of  the 
nervous  system. 

The  Frequency  of  Nervous  Involvement  in  the  Early 
Stages. — Naunyn  from  the  consideration  of  45  cases  of  his 
own,  and  209  collected  from  the  literature,  came  to  the  con- 
clusion that  specific  disease  of  the  nervous  system  appears 
most  frequently  in  the  first  year  after  the  infection.  Ac- 
cording to  this  authority  48  per  cent,  of  all  the  specific  ner- 
vous affections  occur  inside  of  the  first  three  years;  from 
this  time  on  their  frequency  decreases  from  year  to  year. 
This  view  of  Naunyn 's  has  since  often  been  confirmed. 
Gilles  de  la  Tourette  describes  an  unusually  extensive  and 
severe  case  of  syphilis  of  the  brain  and  spinal-cord  mem- 
branes which  developed  two  months  after  the  infection.  The 
same  author  also  reports  a  similar  case  of  Fournier's  in 
which  the  interval  between  the  infection  and  the  manifesta- 
tion of  nervous  syphilis  was  three  months.  Two  of  my 
cases  of  brain  syphilis  developed  four  months  after  the 
infection,  and  in  another  case  three  months  after  the  infec- 
tion, at  the  same  time  that  a  roseola  appeared. 

Late  Development. — Brain  syphilis  may  also  appear  very 
late.  I  have  seen  gummatous  processes  develop  in  the  brain 
after  thirty  years.  Brans  describes  a  case  of  a  large  brain 
gumma  which  developed  forty  years  after  the  infection. 

Patrick  lays  down  this  tenet :  Brain  syphilis  occurs  most 
frequently  during  the  first  year,  then  in  point  of  frequency 
comes  the  second  year,  and  less  frequent  the  third  year. 

50 


NERVOUS  LUES  AND  SPECIFIC  ENDARTERITIS      51 

After  ten  years  its  occurrence  is  the  exception.  In  a  review 
of  185  cases  taken  from  my  own  hospital  records  and  my 
private  practice,  in  55  the  record  was  lacking  in  this  par- 
ticular, in  83  the  first  symptoms  appeared  within  periods 
varying  from  one  to  ten  years,  and  in  47  cases  still  later. 

One  may  say  in  general  that  brain  lues  can  develop  in 
any  stage  of  syphilis,  and  also  that  the  tertiary  stage  is  by 
no  means  the  favorite  one. 

Syphilis  Acquired  Late  in  Life. — It  has  been  said  that  lues 
acquired  late  in  life  predisposes  to  disease  of  the  nervous 
system.  My  own  observations  in  this  regard  show  merely 
that  a  number  of  patients,  who  contracted  syphilis  in  their 
fifties  and  sixties,  after  a  few  years  became  tabetic.  On 
the  other  hand  I  can  point  to  many  cases  in  healthy  young 
persons  who  became  victims  of  severe  nerve  syphilis. 

In  29  cases  of  brain  syphilis  autopsied  by  Neumann  their 
occurrence  with  reference  to  age  was  as  follows : 

Between     1-18  years  of  age 1  case 

20-29  years  of  age 3  cases 

30-39  years  of  age 8  cases 

40-49  years  of  age 4  cases 

50-59  years  of  age 6  cases 

60-69  years  of  age 3  cases 

70-79  years  of  age 2  cases 

My  own  185  cases  occurred  in  point  of  age  in  the  follow- 
ing manner : 

Between    1-10  years  of  age 3  cases 

10-20  years  of  age 2  cases 

20-30  years  of  age 25  cases 

30-40  years  of  age 75  cases 

40-50  years  of  age 55  cases 

50-60  years  of  age 23  cases 

60-70  years  of  age 2  cases 

Men  seem  to  be  more  often  affected  than  women,  which 
may  be  due  to  the  fact  that  men  are  more  exposed  to  in- 
fluences injurious  to  the  nervous  system.  Among  my  185 
cases  125  were  males  and  60  females. 


52  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

It  is  doubtless  true,  as  Lochte  from  material  in  Engel- 
Eeimer's  clinic  demonstrated,  that  syphilis  when  acquired 
late  in  life  is  apt  to  run  an  unusually  severe  course.  Leudet 
reports  two  cases  in  old  people,  in  both  of  whom,  three 
months  after  the  initial  lesion,  symptoms  of  brain  syphilis 
developed. 

Syphilitic  Virus  with  Special  Toxicity  for  the  Nervous 
System. — The  question  as  to  whether  different  kinds  of  spe- 
cific virus  exists,  and  whether  a  certain  form  of  the  toxin 
might  exhibit  a  special  predilection  for  damaging  the  ner- 
vous system,  has  been  much  discussed.  One  may  utilize 
as  an  analogy  the  affinity  of  lead  for  the  motor  tract  and 
ergot  for  the  posterior  columns.  A  certain  number  of 
observations  incline  to  raise  this  question.  In  the  first  place 
the  observations  of  the  occurrence  of  tabes  and  taboparesis 
in  infantile  and  juvenile  persons  are  continually  increasing 
whose  parents  have  had  syphilis,  but  in  whom  no  postluetic 
symptoms  were  demonstrated. 

In  a  long  series  of  observations  the  symptoms  of  nervous 
disease  appeared  in  the  early  years  of  life,  but  never  during 
the  nursing  age,  and  in  all  the  cases  other  etiological  factors, 
such  as  alcohol,  occupation,  worry,  physical  injuries,  and 
sexual  excesses,  which  have  been  considered  either  as  pre- 
disposing causes  or  in  themselves  responsible  for  the  de- 
velopment of  these  diseases  could  be  absolutely  excluded.  In 
all  of  the  cases  the  specific  virus  appeared  as  the  only  factor. 
Second.  The  observations  in  regard  to  the  development 
of  the  syphilogenetic  diseases  in  married  persons  have  also 
increased.  Many  cases  of  both  conjugal  tabes  and  tabo- 
paresis have  been  reported. 

Third.  The  statistics  in  regard  to  a  combination  of  tabes 
and  taboparesis  with  such  specific  conditions  as  cerebro- 
spinal  lues,  syphilitic  spinal  paralysis,  acute  and  chronic 
meningomyelitis  syphilitica  in  married  individuals  have 
likewise  been  augmented.  Such  cases,  for  example,  where 
the  husband  developed  general  paresis,  the  wife  cerebro- 
spinal  lues;  the  husband  tabes,  the  wife  specific  spinal 
paralysis. 

In  this  group  the  influence  of  heredity  and  social  environ- 


NERVOUS  LUES  AND  SPECIFIC  ENDARTERITIS      53 

merit  cannot  be  made  use  of,  since  in  husband  and  wife  they 
were  different.  Their  different  occupations,  the  husband  in 
his  business,  the  wife  in  her  household,  cannot  be  seriously 
considered,  since  both  would  naturally  lead  entirely  differ- 
ent lives.  Syphilis  then  must  be  regarded  as  the  only  causa- 
tive factor  in  the  production  of  these  conjugal  affections 
of  the  nervous  system. 

In  a  case  of  Mendel's  a  luetic  husband  infected  his  wife. 
He  died  of  paresis.  The  wife  married  again  and  infected 
her  second  husband,  and  man  and  wife  became  tabetic.  In 
a  case  of  my  own  the  patient  who  had  been  infected  devel- 
oped tabes  six  years  afterwards,  and  the  person  who  com- 
municated the  infection  to  him,  eight  years  afterwards  be- 
came tabetic.  These  patients  had  not  lived  together  since 
the  infection  of  the  first  one. 

Yet  more  striking  is  the  group  of  cases  which  show  a 
tendency  for  the  appearance  of  tabes,  paresis,  and  tabo- 
paresis  in  families.  Numerous  cases  of  this  nature  have 
been  reported.  We  also  recognize  that  other  syphilogenetic 
diseases  of  the  nervous  system  besides  tabes  and  tabo- 
paresis  may  have  a  tendency  to  occur  in  families.  Remak 
reports  a  mother  with  lues  cerebri,  the  child  with  tabes; 
Nolan,  the  father  brain  syphilis,  the  child  paresis;  Nonne, 
the  father  brain  syphilis,  the  son  tabes;  Skorzynski,  the 
father  brain  syphilis,  the  mother  and  child  paresis.  Such 
observations  might  be  continued  indefinitely.  All  of  the 
cases  speak  against  the  assumption  of  the  social  environ- 
ment as  a  factor,  and  in  favor  of  the  specific  toxin  itself. 
Individual  cases  in  the  literature  also  argue  against  the  etio- 
logical  importance  of  heredity.  The  following  case  is  illus- 
trative of  this : 

A  mother  before  her  infection  had  a  child  who  remained 
healthy.  She  acquired  syphilis  and  became  tabetic,  married 
a  third  husband,  by  whom  she  had  two  children,  and  both 
of  them  developed  paresis. 

There  is  still  another  class  of  cases  which  etiologically 
are  important.  They  are  those  cases  in  which  a  number  of 
persons  have  become  infected  from  the  same  source,  who 
naturally  have  an  entirely  different  heredity  and  social 


54  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

environment,  and  yet  all,  or  nearly  all,  have  developed  dis- 
ease of  the  nervous  system.  A  great  number  of  such  cases 
have  been  reported.  I  have  been  able  to  collect  1902  of  these 
observations  from  the  literature.  The  following  case  will 
illustrate  the  nature  of  them : 

Three  men,  who  were  friends,  one  after  another  had 
intercourse  with  a  syphilitic  puella  during  the  same  night. 
One  of  the  three  developed  tabes,  the  other  two  paresis. 

Two  merchants  acquired  lues  from  the  same  person. 
One  had  later  a  severe  brain  syphilis,  the  other  a  paraplegia 
syphilitica  spinalis. 

My  personal  opinion  formed  from  a  large  experience 
in  cases  where  both  husband  and  wife  became  either  tabetic 
or  paretic  is  that  social  environment  cannot  be  considered 
an  important  factor,  also  that  heredity  and  family  predis- 
position may  be  regarded  as  not  of  much  influence. 

Objections  to  the  Theory  of  a  Special  Toxicity. — However, 
objections  have  been  raised  to  this  view.  Heubner  asks, 
first,  why,  if  the  toxin  itself  is  to  be  regarded  as  the  chief 
etiological  cause,  some  members  of  the  same  group  remain 
free  from  involvement  ?  Another  question  which  he  ^pro- 
pounds is :  Can  one  speak  of  lues  nervosa,  when  in  the  same 
cadaver  one  finds  the  degenerations  of  tabes  or  paresis 
along  with  gummatous  brain  syphilis?  He  argues  that 
gummatous  disease  of  the  brain  and  spinal  cord  does  not  in 
any  sense  indicate  a  specific  nerve  poison,  since  gummata 
are  found  everywhere  in  the  various  organs  of  the  body. 
At  the  same  time  he  refers  to  those  cases  in  his  own  experi- 
ence where  one  of  a  married  couple  became  diseased,  either 
with  a  syphilitic  or  metasyphilitic  affection  of  the  nervous 
system,  and  the  other  one  with  visceral  syphilis. 

Ehrmann's  Theory. — Recently  Ehrmann  has  reported  a 
case  which  is  perhaps  of  great  importance  in  this  connection. 
He  has,  in  the  pursuit  of  his  studies  over  the  probable  path 
of  the  specific  toxin  from  the  initial  lesion  in  the  prepuce, 
devoted  considerable  attention  to  the  nerves.  He  has  found 
in  two  cases  where  the  prepuce  was  extensively  ulcerated 
and  excoriated,  in  a  large  number  of  nerves  of  the  skin  and 
subcutis,  spirochaetes  in  vast  numbers.  Ehrmann  found  the 


NERVOUS  LUES  AND  SPECIFIC  ENDARTERITIS      55 

spirocliaete  not  only  in  the  connective  tissue  accompanying 
the  nerve  fasciculus  and  in  the  lymph  spaces  of  the  nerve 
sheath,  but  also,  to  his  surprise,  in  the  nerve  fasciculus 
itself  between  the  nerve  fibres.  In  the  space  between  the 
nerve  fasciculus  and  the  nerve  sheath,  which,  during  life,  is 
filled  with  lymph,  spirochaetes  lay  entangled  in  irregular 
masses  and  clumps,  also  surrounding,  in  ring-like  for- 
mation, the  nerve  bundle.  In  the  interior  of  the  nerve  they 
were  imbedded  directly  between  the  nerve  fibres.  A  pene- 
tration into  the  medullary  sheath  could  not  be  demon- 


FIG.  27. — (Ehrmann.) 

strated,  the  axis  cylinders  (silver  stain)  were  intact,  symp- 
toms of  inflammation  were  not  discovered  in  the  nerve 
fasciculus. 

Ehrmann  raises  the  question  whether,  analogous  to  the 
clinical  assumption  of  an  ascending  neuritis  in  leprosy  and 
rabies,  there  might  be  found  here  an  explanation  for  the 
origin  of  tabes  with  reference  both  to  the  fact  that  tabes 
almost  exclusively  begins  in  the  lowest  part  of  the  spinal 
cord,  and  also  that  the  spirochsetes  can  remain  latent  for  a 
long  time  and  then  again  resume  their  pernicious  activity. 

In  the  consideration  of  this  theory  one  must  not  forget, 
first,  that  cases  of  tabes  cervicalis  must  then  depend  upon 
initial  lesions  in  the  region  of  the  face  and  head,  and  second, 
in  tabes  neither  in  the  spinal  fluid  in  life  nor  in  the  tissues 


56  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

of  the  spinal  cord  and  its  membranes  have  spirochaetes  ever 
been  found.  Nevertheless  the  theory  of  regarding  the  ana- 
tomical position  of  Schaudinn's  spirochaete  in  the  nerve 
itself  as  an  explanation  of  the  development  of  specific 
nervous  disease  is  an  attractive  one. 

The  Influence  of  Treatment. — It  has  often  been  stated  that 
a  not  thorough  or  an  insufficient  antispecific  treatment  is  the 
cause  of  specific  disease  of  the  circulatory  part  of  the  central 
nervous  system.  The  practical  and  exceedingly  important 
question,  what  value  and  significance  the  intensity  and 
thoroughness  of  treatment  in  the  primary  and  secondary 
stages  of  syphilis  has  towards  the  prevention  of  a  later 
involvement  of  the  nervous  system,  whether  it  be  true  syph- 
ilitic or  metasyphilitic  disease,  deserves  serious  considera- 
tion. Erb  called  attention  in  1900  to  the  fact  that  the 
majority  of  the  cases  of  tabes  follow  an  apparently  light 
infection,  and  for  this  reason  are  apt  to  escape  energetic 
treatment.  Neisser  collected  445  cases  of  tabes  out  of  the 
literature  and  found  that  254  of  them,  57  per  cent.,  had 
never  received  any  anti syphilitic  treatment.  The  same 
opinion,  that  is,  that  those  individuals  are  most  apt  to  de- 
velop syphilitic  or  metasyphilitic  disease  of  the  nervous 
system  who  have  not  been  treated  or  only  insufficiently 
treated,  is  held  by  such  syphilographers  as  Fournier, 
Koposi,  Neumann,  and  by  such  neurologists  as  Heubner, 
Gilbert,  and  Kuh. 

In  striking  contrast  to  this  opinion  is  the  view  expressed 
by  Collins,  of  New  York,  who  from  the  study  of  96  cases 
of  tabes  comes  to  the  conclusion  that  a  thorough  treatment 
of  syphilis  neither  prevents  nor  postpones  the  development 
of  syphilitic  or  metasyphilitic  nervous  disease,  and  in  cases 
not  thoroughly  treated  such  disease  is  apt  to  develop  later 
rather  than  earlier. 

Eulenburg  in  27  cases  of  tabes  found  that  50  per  cent,  of 
them  had  not  been  treated,  40.7  per  cent,  had  undergone  one 
course  of  treatment,  and  only  11  per  cent,  had  taken  a  series 
of  treatment.  Dinkier  reports  49  patients  with  tabes,  in 
which  28.6  per  cent,  had  received  no  treatment,  57  per  cent, 
one  course  only,  and  12.2  per  cent,  a  number  of  courses. 


NERVOUS  LUES  AND  SPECIFIC  ENDARTERITIS      57 

Kron,  as  a  result  of  his  observation  of  tabes  among  women, 
came  to  the  conclusion  that  antispecific  treatment  has  no 
value  in  the  prevention  of  nervous  disease. 

In  my  own  experience  I  have  been  able  to  find  cases 
which  received  a  thorough  and  energetic  treatment,  not  only 
immediately  after  the  infection,  but  also  at  every  secondary 
outbreak,  and  in  two  cases  which  from  the  time  of  the  infec- 
tion on  took  almost  uninterrupted  treatment,  yet  within 
a  period  of  two  years  after  the  date  of  the  infection  devel- 
oped nervous  disease. 

In  35  cases  of  paresis  which  came  under  Schuster's 
observation  there  were  three  which  had  never  received  any 
antispecific  treatment  (8.6  per  cent.),  21  had  taken  only  one 
course  of  treatment  (60  per  cent.),  five  cases  (14.3  per  cent.) 
two  courses,  and  six  (17.1  per  cent.)  received  from  three  to 
nine  courses  of  antispecific  treatment. 

The  specific  infection  was  admitted  in  76  cases  of  cere- 
brospinal  lues.  Among  these  10  were  never  treated  (13.2 
per  cent.),  36  patients  (47.4  per  cent.)  had  one  course,  15 
patients  (19.7  per  cent.)  two  courses,  and  15  patients  re- 
ceived from  three  to  six  courses  of  treatment.  Schuster 
was  unable  to  distinguish  any  difference  in  the  clinical  pic- 
ture in  cases  which  had  been  previously  treated  and  those 
which  had  not  been.  He  describes  several  cases  in  which  the 
most  thorough  treatment  had  been  given  and  yet  these  cases 
presented  the  severest  symptoms  from  the  side  of  the  ner- 
vous system.  The  average  period  of  latency  in  the  un- 
treated and  partially  treated  cases  was  of  no  shorter  dura- 
tion than  in  the  cases  which  had  received  thorough  treat- 
ment. iSchuster  also  concluded  from  his  statistics  that 
those  who  had  received  one  course  of  treatment  became 
tabetic  later  than  those  who  had  received  two  courses,  and 
those  again  developed  tabes  later  than  the  ones  who  had 
taken  a  number  of  courses  of  treatment. 

The  period  of  latency  in  Schuster's  76  cases  of  cerebro- 
spinal  lues,  in  all  of  which  lues  had  been  admitted,  aver- 
aged between  ten  and  eleven  years.  Adding  now  to  these 
76  cases  24  cases  where  the  patients  admitted  having  had 
a  soft  chancre  only,  or  denied  venereal  infection  entirely,  the 


58  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

total  number  of  untreated  cases  would  be  34.  The  average 
period  of  latency  for  these  34  untreated  was  12.2  years — 
a  little  longer  period  than  the  average  for  the  treated  cases. 

Schuster  also  came  to  the  conclusion  that  it  has  not  been 
proven  that  previous  mercurial  treatment  is  able  either  to 
prevent  or  retard  the  development  of  syphilitic  or  meta- 
syphilitic  nervous  disease. 

Two  of  my  own  observations  seem  to  be  pertinent  here : 

A  young  officer,  who  had  received  no  treatment  for  his 
primary  and  secondary  symptoms,  four  months  after  his 
infection  developed  brain  syphilis,  from  which  he  died  four 
months  later.  In  another  case  because  of  an  unusually 
severe  primary  lesion  the  antispecific  treatment  was 
administered  energetically  and  thoroughly,  yet  during  this 
treatment  symptoms  of  basilar  meningitis  with  psychic 
disturbances  appeared. 

Oppenheim  takes,  however,  a  somewhat  different  view 
of  the  question.  He  says  that  in  two  individuals  who  have 
been  infected,  other  things  being  equal,  the  one  who  has  re- 
ceived a  thorough  antispecific  treatment  is  to  a  lesser  extent 
threatened  with  a  brain  affection  than  the  one  who  has 
received  insufficient  treatment  or  no  treatment  whatever. 

Eecently  Mattauscheck  (Vienna),  after  a  careful  study 
of  this  question  among  Austrian  officers  who  furnished  the 
opportunity  for  continuous  observation  over  a  long  period 
of  years,  came  to  the  conclusion  that  those  who  had  received 
thorough  treatment  stood  the  best  chance  of  remaining  free 
from  nervous  involvement. 

In  only  one  thing  do  all  the  authorities  on  this  subject 
seem  to  be  able  to  agree.  This  is,  from  these  observations 
one  must  not  draw  the  conclusion  that  syphilis  should  not 
always  be  thoroughly  treated. 

The  Influence  of  the  Severity  of  the  Course  on  Nervous 
Involvement. — The  opinion  first  expressed  by  Broadbent, 
Fournier,  and  Erb,  and  later  confirmed  by  many  other 
observers,  that  brain  syphilis,  and  especially  disease  of  the 
arteries  of  the  brain,  was  apt  to  occur  in  cases  where  the 
primary  and  secondary  stages  were  mild,  has  much  in  its 
favor.  I  can  by  no  means,  however,  confirm  this  view  in  all 


NERVOUS  LUES  AND  SPECIFIC  ENDARTERITIS      59 

of  my  cases.  A  number  of  my  cases  ran  a  severe  course 
from  the  beginning,  the  primary  lesion  took  on  a  malignant 
character,  the  eruption  instead  of  being  macular  was  papu- 
lar and  pustulopapular,  and  new  exacerbations  occurred 
during  antispecific  treatment.  Oppenheim  also  reports 
brain  syphilis  after  both  mild  and  severe  primary  and 
secondary  symptoms. 

The  Influence  of  Extragenital  Infection. — Extragenital  in- 
fection does  not  bear  any  special  significance  to  brain  syph- 
ilis. Halm  reports  307  cases  of  extragenital  infection,  and 
in  none  of  these  cases  did  syphilis  of  the  brain  occur. 

The  Influence  of  Head  Injuries. — Considerable  has  been 
written  concerning  the  influence  of  head  injuries  in  the 
development  of  brain  syphilis.  My  own  observations  in  this 
regard  lead  me  to  the  belief  that  even  here  we  should  not 
overestimate  the  influence  of  trauma.  Every  year  at  the 
Eppendorf  Hospital  I  have  the  opportunity  of  observing 
about  150  more  or  less  severe  injuries  of  the  head,  and 
among  these  patients  there  is  a  fair  proportion  who  have 
acquired  syphilis.  I  can  recall  only  two  cases  where  symp- 
toms of  brain  lues  developed  after  trauma. 

Bailey  from  his  observations  comes  to  the  conclusion 
that  while  one  a  priori  cannot  deny  the  creation  of  a  locus 
minoris  resistentia  through  trauma,  nevertheless  syphilis 
of  the  brain  occurs  extremely  seldom  after  injuries  of  the 
head. 

The  Influence  of  Mental  Work. — It  has  been  asserted  and 
also  disputed  that  the  brain  worker  is  more  susceptible  to 
brain  syphilis  than  the  man  who  works  with  his  hands.  In  a 
review  of  my  material,  both  in  the  hospital  and  in  my  pri- 
vate practice,  I  am  not  able  to  discover  any  more  frequent 
occurrence  of  brain  syphilis  in  the  brain  worker  than  in  the 
ordinary  laborer.  Tarnowsky  in  100  cases  of  cerebral  lues 
was  able  to  demonstrate  only  six  cases  in  which  the  causa- 
tive factor  appeared  to  be  intellectual  overactivity. 

Psychic  trauma  and  acute  infectious  diseases  may  also 
be  regarded  as  of  insignificant  influence  as  causative  factors. 

The  Influence  of  Alcohol. — On  the  other  hand,  alcohol 
predisposes  a  syphilitic  patient  to  an  outbreak  of  brain 


60  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

syphilis.  Among  my  cases  of  brain  lues  chronic  alcoholism 
often  exists.  Lochte  in  his  excellent  monograph  on  this 
subject  says  that  syphilis  in  alcoholics  often  is  a  syphilis 
gravis.  Tarnowsky's  statistics  makes  clear  the  role  which 
alcohol  plays  in  lues  cerebri.  In  100  patients  with  syphilis 
of  the  brain,  he  finds  45  who  were  habitual  drunkards. 
However,  it  should  be  stated  that  the  organ  most  often 
affected  in  alcoholics  with  syphilis  is  not  the  brain  but  the 
liver. 

Neuropathic  Heredity. — The  etiological  importance  of  a 
congenitally  weak  nervous  system  should  also  be  considered. 
Oppenheim,  Fournier  and  Lamy  all  regard  a  poor  nervous 
inheritance  as  having  some  influence  in  the  causation  of 
parasyphilitic  diseases.  In  185  cases  of  lues  cerebralis, 
spinalis  and  cerebrospinalis  there  was  in  24  no  history 
regarding  heredity,  in  136  a  good  family  history,  and  in 
25  a  poor  one. 

Symptomatology  of  the  Arteritic  Form  of  Cerebral  Syphilis. 
— We  come  now  to  the  symptomatology  of  brain  lues  and 
will  first  consider  the  clinical  symptoms  of  the  arteritic 
form.  Among  the  manifold  symptoms  which  the  pathology 
of  syphilis  produces,  one  is  struck  with  the  frequency  of 
the  occurrence  of  symptoms  of  a  monoplegic  and  hemiplegic 
nature.  We  have  previously  called  attention  to  the  fact 
that  the  blood-vessels  suffer  not  only  because  of  genuine 
syphilitic  disease,  but  also  secondarily  through  the  involve- 
ment of  their  environment,  the  nervous  tissue  itself  as  well 
as  the  brain  coverings,  and  that  in  either  way  lumen  closure 
may  be  brought  about  with  the  consequent  result  to  the 
nerve  tissue  of  softening  and  necrosis. 

Although  the  various  specific  processes  are  found  very 
often  in  brain  involvement,  indeed  most  often  in  combina- 
tion, nevertheless  the  clinical  manifestation  of  brain  syph- 
ilis in  the  form  exclusively  of  Heubner's  arteritis  not  infre- 
quently occurs. 

The  Time  of  Occurrence. — All  observers  agree  that  the 
affection  of  the  arteries  may  occur  soon  after  the  specific 
infection.  A  great  number  of  statistics  exist  on  this  point. 
We  find  in  these  statistics  cases  where  half  a  year  after  the 


NERVOUS  LUES  AND  SPECIFIC  ENDARTERITIS     61 

infection  a  paralysis  referable  to  lues  appeared.  Kahler 
reports  a  softening  of  the  pons  as  the  result  of  an  arteritic 
basilar  thrombosis  in  a  patient  whose  primary  lesion  was 
not  yet  entirely  healed.  On  the  other  hand  there  are  cases 
where  a  specific  paralysis  develops  years  after  the  infection. 
In  such  cases,  we  must  consider  whether  we  have  to  deal, 
with  a  specific  arterial  disease  or  with  the  ordinary  arterial 
sclerosis. 

Growers  in  an  analysis  in  56  cases  of  specific  hemiplegia 
found  that  the  paralysis  in  one-fourth  of  the  cases  occurred 
within  the  first  two  years  after  the  infection ;  the  other  cases, 
with  the  exception  of  two,  one  of  which  took  place  18  and 
the  other  19  years  afterwards,  were  distributed  within  a 
period  of  12  years  from  the  date  of  the  infection.  In  one 
case  of  Gowers's  series  the  paralysis  appeared  three 
months  after  the  infection. 

According  to  Fournier  the  arteritic  hemiplegia  occurs 
most  often  between  the  sixth  and  tenth  year. 

My  own  cases  of  arteritis  luetica  show  a  wide  difference 
in  the  time  of  occurrence,  varying  from  7  months  to  27 
years,  the  greater  number  occurring  in  the  fifth,  sixth,  and 
seventh  years  after  the  primary  lesion. 

Age  of  the  Patients. — The  age  of  the  patients  ranged,  in 
the  women  from  24  to  56  years,  in  the  men  from  18  to  55 
years.  Both  Siemerling  and  Gowers  have  reported  cases  of 
endarteritic  paralysis  in  children  who  were  afflicted  with 
hereditary  lues. 

Prodromal  Symptoms. — In  many  cases  the  attack  is  pre- 
ceded by  prodromal  symptoms.  These  are  headache,  dizzi- 
ness, insomnia,  changes  in  the  disposition  in  the  form  of 
irritability,  loss  of  ambition,  incapacity  for  continued  men- 
tal effort,  and  impaired  memory.  These  symptoms  must 
not,  however,  be  considered  as  characteristic  of  specific 
endarteritis ;  they  may  also  appear  in  other  manifestations 
of  brain  syphilis.  The  triad,  headache,  dizziness,  and  failure 
of  memory,  may  be  regarded  as  a  prodromal  symptom- 
complex  in  arteriosclerosis,  cerebral  apoplexy  and  brain 
atrophy.  One  must  further  add  that  both  light  and  severe 


62  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

attacks  of  apoplexy  occur  not  infrequently  without  the 
slightest  warning  in  the  form  of  prodromal  symptoms. 

The  knowledge  that  these  symptoms  are  often  precur- 
sory of  severe  manifestations  of  arteritis  is  extremely  im- 
portant, because  they  represent  danger  signals  and  because 
a  timely  and  efficient  therapy  is  able  to  prevent,  under  favor- 
able circumstances,  the  development  of  later-coming  brain 
lesions. 

Headache. — The  headache  may  be  very  severe  and  is 
usually  located  on  different  parts  of  the  head ;  it  is  seldom 
localized.  It  is  most  often  diffuse  in  character  and  may  be 
described  as  dull  and  stupefying,  rather  than  as  boring  and 
agonizing.  It  is  sometimes,  though  not  always,  worse  by 
night  than  by  day.  It  is  seldom  constant,  and  when  inter- 
mittent is  apt  to  be  more  severe.  It  may  disappear  for 
weeks  and  months,  only  to  reappear  again  without  any 
apparent  cause. 

Headache  may  remain  for  a  long  time  the  only  symptom, 
and  it  is  advisable  in  all  cases  where  the  patient  complains 
of  severe  headache  for  which  no  cause  can  be  discovered  to 
at  least  suspect  syphilis. 

Dizziness. — Dizziness  occurs  frequently  in  short  attacks, 
but  may  also  be  more  or  less  permanent  in  character.  Like 
headache  it  may  be  the  only  symptom  for  a  long  time,  but 
monosymptomatic  dizziness  is  far  less  frequent  than  mono- 
symptomatic  headache.  As  a  rule  the  dizziness  is  lessened 
when  the  patient  lies  down. 

Insomnia. — The  insomnia  may  be  transient  or  persistent 
in  nature.  Either  the  patient  awakes  in  the  morning  unre- 
freshed  from  his  sleep  or  is  absolutely  unable  to  sleep  at  all. 

Tschisch  calls  attention  to  the  relative  frequency  of  sud- 
denly appearing  and  intractable  insomnia  in  the  beginning 
of  brain  syphilis,  which  he  considers  especially  important 
for  the  diagnosis  where  symptoms  of  neurasthenia  are  lack- 
ing. It  appears  to  me  doubtful  whether  the  thickening  of 
the  temporal  artery  in  the  early  stages  is  as  frequent  as 
Tschisch  would  have  us  think ;  he  finds  it  in  every  case. 

Psychic  Disturbances. — Among  the  psychic  symptoms  the 
one  most  often  present,  and  for  the  patient  the  most  dis- 


NERVOUS  LUES  AND  SPECIFIC  ENDARTERITIS      63 

turbing,  is  the  impaired  capacity  for  work.  Also  the  abnor- 
mal irritability  is  apt  to  be  a  most  annoying  symptom ;  the 
loss  of  memory  is  less  intense  in  character.  In  some  cases 
the  loss  of  intelligence,  the  lack  of  general  and  special 
interest,  makes  the  patient  dull  and  stupid.  Add  to  these 
symptoms  a  tendency  to  weep,  and  the  clinical  picture  pre- 
sented causes  one  to  think  of  the  dementia  from  general 
paresis.  In  rare  cases  the  only  general  symptom  may  be 
a  disturbance  of  speech  in  the  form  of  bradylalia. 

Intoxicated-like  State  of  Heubner. — Heubner  describes  a 
condition  somewhat  resembling  an  intoxicated  state,  a  form 
of  semiconsciousness.  The  same  condition  has  also  been 
described  by  Rumpf  and  Oppenheim.  The  patients  are  able 
to  do  all  sorts  of  things,  while  in  this  semiconscious  state, 
but  a  remembrance  of  the  same  is  lacking. 

Periods  of  excitement  may  alternate  with  periods  of 
apathy.  In  other  cases  there  occur  longer  or  shorter  attacks 
of  falling  asleep.  A  subsidence  of  this  condition  is  possible, 
but  it  should  always  be  regarded  as  a  severe  symptom,  be- 
cause it  is  often  a  forerunner  of  a  severe  paralysis  or  of  a 
coma  preceding  death.  The  following  case  illustrates  this 
condition : 

A  woman  fifty-six  years  old,  from  whom  after  the  most 
painstaking  inquiry  no  history  of  syphilis  could  be  obtained, 
was  sent  to  the  hospital  by  an  experienced  physician,  with 
the  diagnosis  delirium  alcoholica  incipiens,  in  spite  of  the 
fact  that  no  evidence  of  potus  could  be  adduced.  The  objec- 
tive symptoms  were  hypera3mia  of  the  optic  discs  with  indis- 
tinctness of  their  borders,  increased  activity  of  the  tendon 
reflexes,  and  a  diffuse  sensitiveness  of  the  head  to  percus- 
sion. The  patient  had  hallucinations,  lay  half  sleeping  and 
gave  only  partial  and  incorrect  responses  to  questioning, 
her  recollection  of  events  was  poor,  and  when  completely 
awake  she  talked  at  times  in  a  foolish  manner.  She  now 
and  then  busied  herself  with  some  work  without  being  fully 
conscious  of  what  she  was  doing,  and  later  had  no  recollec- 
tion of  what  she  had  done.  In  spite  of  energetic  antispecific 
treatment  she  did  not  improve  and  a  week  later  died.  The 


64 


SYPHILIS  AND  THE  NERVOUS  SYSTEM 


autopsy  showed  an  extensive  disseminated  endarteritis  and 
periarteritis  gummosa. 

There  are  many  cases  in  which  general  symptoms  only, 


FIG.  28. — Arteries  of  the  brain-base.  (Monakow,  "Brain  Pathology" ;  Nothnagel's  " Special 
Pathology  and  Therapy,"  vol.  ix.)  T,  separated  temporal  lobes;  a.  cer.  a.,  arteria  cerebri  an- 
terior; con.  ant.,  arteria  communicans  anterior;  c.  p.,  arteria  communicans  posterior;  a.  /.  Sy., 
arteria  fossse  sylvii;  a.  lent.,  arteria  lenticularis;  a.  ch.  a.,  arteria  choroidea  anterior;  a.  c.  p., 
arteria  cerebri  profunda;  a.  I.,  arteria  temporalis;  a.  occ.,  arteria  occipitalis;  o.  baa.,  arteria 
basilaris;  a.  cer.  sup.,  arteria  cerebelli  superior;  a.  cer.  med.,  arteria  cerebelli  media;  a.  cer.  inf., 
arteria  cerebelli  inferior;  a.  ver.,  arteria  vertebralis;  s.  p.  a.,  arteria  spinalis  anterior;  I-XII, 
cranial  nerves;  1-5,  cortical  branches  of  the  fossae  sylvii. 

such  as  irritability,  inability  for  mental  concentration,  and 
loss  of  memory,  are  present,  and  localized  symptoms  appear 
either  late  or  not  at  all.  In  this  class  of  cases  one  is  more 


NERVOUS  LUES  AND  SPECIFIC  ENDARTERITIS      65 


inclined  to  think  of  a  psychosis  or  brain  tumor.  These  cases 
are  not  infrequently  unresponsive  to  specific  treatment. 
Cases  may  also  begin  apparently  as  a  severe  apoplexy,  and 
on  postmortem  no  hemorrhage  or  area  of  softening  will  be 
found,  but  only  a  Heubner's  endarteritis. 

Arterial  Disease  on  the  Base  of  the  Brain.  —  We  have  men- 
tioned before  that  Heubner's  arteritis  affects  most  often  the 
arteries  of  the  brain.  We  a 

can  see  at  a  glance  the 
blood  supply  by  consulting 
Fig.  28.  The  large  arte- 
rial trunks  are  represented 
through  the  basilar  ar- 
tery, which  sends  the  large 
branches,  the  arteria  cere- 
belli  superior  and  the  ar- 
teria profunda  cerebri,  to 
the  pons  and  brain  stem. 
There  also  can  be  seen  the 
arteria  fossae  sylvia  which 
supplies  the  centre  of  the 
brain,  through  the  arteria 
corporis  callosi  and  the 
arteria  lenticularis. 


nf 
d, 

that    the 


rmn 
O 


FlG-  29-  —  Arterial  ramification  of  the  pedun- 
cu,ar   region  of   the   brain      Qne   geeg   numeroug 

branches  Siven  off   from  the   arteria  cerebri  pos- 
terior   and    the    arteria   communicans    posterior. 


. 

region,  for  Which      "•  arteria  basilarls;  6,  arteria  cerebelli  superior; 
c,  arteria  cerebri  posterior;  d,  arteria  communi- 

rOCeSSeS     appear      cans  posterior;  e,  carotid  interna;   /,  nervus  ocu- 
,  ,  ,.,        ,.  .          lomotorius;  g,  pons;  h,  cms  cerebri;  t,  lobus  tem- 

tO    ShOW    a,   predilection,    IS      poralis;*-,  chiasmanervorumopticorum;;,  tractus 
.1-1  •  -•     j        •  ,-1  olfactorius. 

richly  provided  with  arte- 

rial supply,  both  from  the  carotid  and  ba,silar  arteries. 

The  arterial  relations  have  been  carefully  studied  by 
Heubner  and  Bumpf.  The  arteries  which  are  given  off  at 
right  angles  from  the  arteria  fossae  sylvii  are  end  arteries, 
in  the  sense  of  Cohnheim,  that  is,  the  brain  region  supplied 
by  them  has  no  possibility  of  a  collateral  blood  supply.  This 
explains  why  so  often  diseases  of  the  basilar  arteries  pre- 
sent the  same  clinical  picture:  hemiplegia  on  the  one  hand 
and  symptoms  of  pons  affection  on  the  other. 

5 


66  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

Variations  in  Blood-pressure. — There  is  not  always  a  com- 
plete shutting  off  of  the  vessel  lumen,  but  very  often  only  a 
partial  obstruction  to  the  passage  of  the  blood  exists,  de- 
pending on  the  degree  of  narrowing  in  the  arterial  lumina. 
Where  such  conditions  exist  we  naturally  find  variations  in 
the  blood  pressure.  The  arteries  on  the  distal  side  of  the 
narrowed  lumina  would  be  subjected  to  less  pressure  and 
thus  receive  a  diminished  blood  supply,  and  then  gradually 
later  on  would  become  filled  with  blood  again. 

This  variation  in  blood-pressure  and  consequent  dis- 
turbance of  nutrition  is  the  anatomical  explanation  of  the 
attacks  of  dizziness  as  well  as  the  transient  paralyses  so 
often  observed.  The  transient  paralysis  may  last  only  a 
few  minutes,  but  can  persist  for  several  hours  and  even 
longer.  Paraesthesias  appear  along  with  the  paralysis,  and 
it  not  infrequently  happens  that  the  disturbance  stops  with 
the  paraesthesia  and  a  genuine  paralysis  does  not  develop. 
Either  only  one  extremity  is  affected  or  an  upper  and  lower 
extremity,  or  these  together  with  the  face  and  speech.  It 
frequently  happens  that  the  speech  alone  is  affected  or  the 
speech  in  combination  with  panesthesias  in  the  face  and 
tongue. 

Symptoms  of  Motor  Irritation. — The  transient  circulatory 
disturbances  may  also  produce  symptoms  of  motor  irri- 
tation. We  observe  then  the  extremities  attacked  with  light 
clonic  contractions  without  any  clouding  of  the  conscious- 
ness. Sometimes  one  sees  one  extremity  after  the  other 
become  paralyzed.  In  the  course  of  a  day  the  gradually  in- 
creasing thrombosis  in  the  artery  may  lead  to  an  ascending 
or  descending  complete  hemiplegia,  while  at  the  same  time 
the  consciousness  remains  absolutely  intact. 

Transient  Paralysis. — Transient  paralyses  often  recur,  it 
may  be  after  days,  weeks,  or  months.  In  some  cases  they 
may  still  remain  transient,  in  others  after  several  recur- 
rences become  more  or  less  permanent.  A  long  existing 
paralysis,  which  has  almost  entirely  receded  under  anti- 
syphilitic  treatment^  may  also  return  after  months  and 
years  have  passed,  and  in  the  same  part  of  the  body.  This, 
to  a  certain  extent,  is  characteristic  of  a  syphilitic  paralysis. 


NERVOUS  LUES  AND  SPECIFIC  ENDARTERITIS      67 

It  is,  moreover,  easy  to  understand  when  one  considers  that 
the  anatomical  changes  once  started  cannot  again  become 
retrogressive,  that  also  in  these  places  where  the  circula- 
tion has  been  restored  through  the  changes  in  the  lumen  a 
strong  predisposition  exists  to  the  formation  of  another 
thrombus.  The  report  of  the  following  cases  will  illus- 
trate this. 

A  laborer  thirty-four  years  old,  four  years  after  a 
specific  infection,  was  received  at  the  Eppendorf  Hospital 
suffering  with  a  right-sided  hemiparesis  which  had  devel- 
oped without  any  accompanying  apoplectic  symptoms. 
Under  antispecific  treatment  he  recovered  in  the  course  of 
about  four  weeks  without  any  residual  paralysis  remaining. 
Eight  months  afterwards  the  patient  was  again  received  at 
the  hospital  on  account  of  severe  headache.  At  the  same 
time  he  had,  intermittently,  clonic  spasms  in  the  right  hand, 
accompanied  by  parsesthesias  and  a  right  hemiparesis.  By 
means  of  large  doses  of  potassium  iodid  his  symptoms  again 
disappeared.  Six  months  later  the  same  patient  presented 
himself  with  a  return  of  his  headaches,  and  in  addition  di- 
plopia  and  paraesthesias  and  weakness  in  the  right  lower 
extremity.  Objectively,  besides  the  paresis  which  had  re- 
turned for  the  third  time,  there  was  a  weakness  of  the  right 
abducens  and  a  mild  double  optic  neuritis.  Under  suitable 
treatment  these  symptoms  again  cleared  up,  leaving  only  a 
partial  optic  atrophy. 

Apoplectic  Symptoms. — The  acutely  developing  hemi- 
plegia  may  appear  accompanied  by  the  symptoms  of  a 
severe  apoplexy,  complete  loss  of  consciousness,  vomiting, 
convulsions,  or  with  mild  symptoms  such  as  light  disturb- 
ances of  the  sensorium,  dizziness,  nausea,  or  indeed  without 
any  of  these  symptoms  whatever.  The  presence  or  absence 
of  these  accompanying  symptoms  cannot  be  regarded  in 
themselves  as  having  any  special  significance  in  distinguish- 
ing between  a  specific  or  non-specific  hemiplegia. 

The  form  of  paralysis  also  does  not  differ  from  the 
paralysis  of  the  ordinary  arteriosclerosis,  except  that  it  is 
not  apt  to  be  so  severe  as  the  latter,  because  hemorrhages 
of  the  specific  variety  are  of  less  frequent  occurrence. 


68  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

One  rarely  gets  a  chance  to  autopsy  a  case  of  syphilitic 
hemiplegia,  because  the  symptoms  usually  disappear  under 
treatment.  Twice,  however,  because  of  complications,  I 
have  had  this  opportunity.  Both  cases  were  in  young  per- 
sons. In  one  case  the  hemiplegia  developed  two  years  after 
the  infection,  in  the  other,  four  years  afterwards.  The 
paralysis  in  one  case  had  disappeared,  in  the  other  a  con- 
dition of  contracture  had  developed.  In  one  case  there  was 
a  small  area  of  necrosis  in  the  internal  capsule,  in  the  other 
a  large  area,  both  due  to  an  arteritis  and  consequent  sten- 
osis of  the  arteria  fossae  sylvii. 

Although  one  is  accustomed  to  attribute  monoplegias 
or  cerebral  facial  paralyses  to  disturbances  in  a  circum- 
scribed portion  of  the  cortex,  numerous  observations  prove 
that  central  lesions  may  also  produce  these  isolated  dis- 
turbances of  cortical  function.  Isolated  convulsions  like- 
wise do  not  necessarily  mean  a  cortical  localization  of  the 
trouble.  The  situation  is  different,  however,  in  convulsions 
appearing  in  a  part  previously  paralyzed. 

The  aphasia  is  usually  a  motor  aphasia,  and  is  in  com- 
bination with  a  monoplegia  or  a  hemiplegia  or  a  paralysis 
of  the  facial  or  of  the  tongue ;  it  may,  however,  appear  as 
the  only  symptom. 

Heilbronner  reports  a  case  of  word  blindness  and  word 
deafness  due  to  a  syphilitic  cause.  The  aphasia  is  frequently 
transient  in  character,  in  some  cases  lasting  a  few  minutes, 
in  others  several  hours,  and  still  others  a  number  of  days. 

Triplegia. — A  relatively  frequent  type  of  syphilitic 
paralysis  is  triplegia,  where  the  paralysis  involves  both 
extremities  on  the  one  side  of  the  body  and  one  extremity  on 
the  other. 

Pons  Involvement. — Another  form  presents  the  clinical 
appearance  of  a  pons  affection.  In  this  form  there  is  in- 
volvement of  the  arteria  basilaris.  Affections  of  the  basilar 
artery  are,  as  a  rule,  serious.  The  following  case  repre- 
sents disease  of  the  basilar  artery. 

A  strong  man  in  the  prime  of  life,  father  of  three  healthy 
children,  after  prodromal  symptoms  in  the  nature  of  head- 
ache of  several  weeks'  duration,  suddenly  became  affected 


NERVOUS  LUES  AND  SPECIFIC  ENDARTERITIS     69 

with  disturbances  of  speech  and  swallowing  and  double 
vision.  Examination  revealed  an  abducens  paralysis,  diffi- 
culty in  swallowing  and  articulation,  and  a  left  hemiplegia. 
Death  occurred  after  three  days.  The  autopsy  demon- 
strated a  marked  endarteritis  of  the  basilar  artery  about 
one-half  cm.  in  length,  with  secondary  processes  of  soften- 
ing in  the  pons.  There  were  also  in  the  various  branches 
of  the  circle  of  Willis  multiple  endarteritic  lesions. 

Sensation. — Sensation  is  only  in  rare  cases  severely 
affected,  and  in  the  majority  of  cases  disturbances  of  it  are 
entirely  lacking. 

Hemianopsia. — Hemianopsia  may  occasionally  be  found 
in  those  cases  where  the  optic  radiations  are  affected  and 
the  lesion  may  occur  anywhere  from  the  chiasm  to  the 
occipital  lobes.  This  symptom  is  met  with  less  often,  how- 
ever, than  in  cerebral  arteriosclerosis  and  brain-tumors. 

The  following  case  is  an  interesting  one  in  this  connec- 
tion :  It  concerns  a  man  27  years  old,  who  eight  years  pre- 
viously had  contracted  syphilis,  taken  a  course  of  inunc- 
tions, and  since  then  had  observed  no  symptoms  of  his  syph- 
ilis. Six  days  before  coming  under  my  care  while  working 
he  was  suddenly  seized  with  a  severe  attack  of  dizziness, 
vomited  several  times,  and  complained  of  severe  pain  in  the 
forehead  and  occiput.  Convulsions  did  not  appear.  He 
observed  soon  afterwards  that  he  could  not  see  towards  the 
left. 

Examination  of  the  field  of  vision  showed  a  left  hemian- 
opsia  with  normal  eye  background  and  normal  pupils. 
There  was  a  slight  weakness  in  the  left  facial  around  the 
angle  of  the  mouth,  also  of  the  right  abducens.  The  two  last 
symptoms  disappeared  in  the  course  of  the  next  seven  days. 
The  extremities  were  not  involved,  nor  was  the  speech.  The 
skull  over  the  right  occipital  lobe  was  tender  to  percussion. 
The  other  organs  were  normal. 

Under  mercurial  treatment  the  headache  and  dizziness 
disappeared,  but  the  hemianopsia  remained.  The  patient 
again  took  up  his  occupation  as  masseur.  Four  years  later 
he  had  another  attack  of  dizziness,  but  not  as  severe  as 
the  first.  Another  careful  examination  revealed  the  same 


70  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

condition  in  the  field  of  vision,  but  no  other  anomalies  on 
the  part  of  the  nervous  system.  The  attacks  of  dizziness 
again  disappeared  and  the  patient  returned  to  work.  Six 
months  later  he  died  of  a  croupous  pneumonia  in  the  right 
lung. 

At  the  autopsy  the  most  of  the  cuneus  and  the  portion  of 
the  precuneus  bordering  on  the  parieto-occipital  fissure  were 
destroyed  and  occupied  by  an  area  of  softening.  The  rest 
of  the  brain  was  normal.  In  the  basilar  artery  as  well  as 
the  arteries  in  the  circle  of  Willis  there  was  found  a  mod- 
erate degree  of  arteriosclerosis.  As  evidences  of  syphilis 
in  other  organs  of  the  body  two  gummatous  nodules  were 
found  in  the  liver  and  in  the  right  testicle  an  indurated 
orchitis. 

Anomalies  of  the  pupils  in  specific  hemiplegia  are  not 
infrequent.  Loss  of  the  pupil  reflexes,  the  loss  of  the  reflex 
to  light,  slowness  of  contraction  of  the  pupil  to  light,  irregu- 
larity of  the  pupils  in  size  and  shape  may  sometimes  be 
observed. 

The  Course. — The  course  of  the  hemiplegia  varies.  It 
may  pass  away  quickly  or  slowly,  it  may  terminate  in  a 
paresis  with  or  without  consequent  increase  of  the  tendon 
and  decrease  of  the  superficial  reflexes,  or  finally  a  con- 
tracture  hemiplegia  may  develop.  The  improvement  in  the 
lower  extremities  is  usually  much  greater  than  in  the  upper, 
as  is  the  case  in  the  ordinary  hemiparesis,  but  sometimes 
the  reverse  is  true :  the  upper  extremity  improves  greatly, 
while  the  lower  lags  behind. 

In  those  cases  in  which  the  paralysis  entirely  regresses 
or  only  a  paresis  remains,  the  remaining  psychical  functions 
may  remain  absolutely  intact  throughout  a  long  life,  always 
provided  of  course  that  syphilis  causes  no  further  damage. 
In  other  cases  the  intelligence  is  disturbed,  the  memory  suf- 
fers and  the  entire  mental  capacity  of  the  patient  sinks  in  a 
marked  degree,  similar  to  the  condition  sometimes  encoun- 
tered in  the  hemiplegia  of  old  age.  In  the  specific  form, 
however,  this  condition  may  also  be  met  with  in  young 
persons.  It  also  happens  that  an  arteritic  hemiplegia  is 
followed  after  a  number  of  years  by  a  general  paresis. 


NERVOUS  LUES  AND  SPECIFIC  ENDARTERITIS      71 

The  speech  disturbances  have  no  points  of  differentiation 
from  aphasia  occurring  in  the  hemiplegia  of  advanced  age. 

The  following  case  representing  an  isolated  facial 
paralysis  in  combination  with  general  psychic  symptoms  is 
interesting: 

A  laborer  thirty-seven  years  old  had  an  initial  lesion 
three  years  before,  he  had  undergone  one  course  of  inunc- 
tions, and  was  temperate  in  the  use  of  alcohol  and  tobacco. 
He  was,  until  his  present  illness,  always  healthy  and  am- 
bitious to  work.  Three  months  previous  to  his  entrance  into 
the  hospital,  without  any  assignable  reason  he  lost  interest 
in  everything  around  him,  was  forgetful,  could  only  with 
difficulty  keep  from  weeping,  laughed  without  cause,  and 
did  nothing  but  sit  and  stare  into  space.  He  had  no  ideas 
of  grandeur  and  did  not  complain  of  headache  or  dizziness. 
The  only  objective  sign  was  a  slight  right-sided  paralysis. 
All  these  symptoms  disappeared  completely  under  a  three 
months'  mixed  treatment. 

Another  case  in  which  the  symptoms  are  entirely  of  a 
psychic  nature  is  also  of  interest  here. 

A  ship's  officer,  thirty-eight  years  old,  was  sent  by  his 
physician  to  the  hospital,  because  recently  his  mental  capa- 
city had  failed  so  rapidly.  He  sat  around  in  a  stupid  man- 
ner, seemed  to  be  unable  to  write,  was  indifferent  and  list- 
less, and  his  memory  was  very  poor.  His  sleep  was  fairly 
good,  but  he  complained  of  a  severe  headache,  which  was 
diffuse  in  character.  He  had  always  been  strong  and 
healthy,  did  not  use  alcohol,  but  six  years  before  had 
acquired  syphilis.  His  secondary  symptoms,  from  his  de- 
scription, had  been  severe  and  he  had  received  thorough 
treatment  for  them.  At  the  time  of  his  entrance  into  the 
hospital,  outside  of  the  old  signs  of  former  secondary 
lesions,  his  only  symptoms  were  a  well-marked  dementia, 
with  none  of  the  earmarks  of  a  paresis,  and  no  objective 
symptoms  from  the  side  of  the  nervous  system,  except  very 
active  tendon  reflexes.  He  soon  developed  a  complete  dis- 
turbance of  consciousness,  had  to  be  fed  with  a  tube,  and 
four  months  later  died  from  decubitus  and  aspiration 
pneumonia. 


72  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

The  autopsy  revealed,  outside  of  a  moderate  arterio- 
sclerosis of  the  ascending  aorta,  no  abnormalities  in  the 
chest  or  abdomen.  The  basal  arteries  of  the  brain  showed 
macroscopically  Heubner's  changes,  microscopically  a 
marked  endarteritis,  meso-  and  peri-arteritis  in  the  trunks 
on  the  base  as  well  as  in  the  arterioles  of  the  cortex.  Ex- 
amination of  the  portions  of  the  cortex  did  not  reveal  the 
changes  in  ganglion  cells  which  are  found  in  paresis. 

Charriere  and  Klippel  have  classified  the  various  possi- 
bilities in  the  termination  of  specific  paralysis  in  the  follow- 
ing manner:  the  ''forme  intellectuelle, "  the  "forme  pare- 
tique  legere  et  aphasique, ' '  the  ' '  forme  paraly tique  grave, ' ' 
and  the  "forme  apoplectique. " 

Diagnosis. — The  diagnosis,  as  a  rule,  is  not  difficult.  If 
a  hemiplegia  occurs  in  an  individual  before  the  age  of  45, 
before  one  expects  to  find  arteriosclerosis,  and  one  is  also 
able  to  exclude  heart  disease  and  disease  of  the  kidneys,  and 
those  affections  such  as  leukaemia,  scurvy,  pernicious 
anaemia  and  alcoholism,  which  lead  to  a  hemorrhagic  dia- 
thesis, and  if  there  exists  no  acute  condition  which  might 
cause  an  embolus,  syphilis  is  naturally  to  be  thought  of  as 
the  cause  of  the  apoplexy.  The  diagnosis  becomes  more 
probable,  of  course,  if  there  is  a  history  of  syphilis.  On 
the  other  hand,  if  there  is  no  history  nothing  is  proven. 
The  majority  of  cases  occur  later  than  the  period  at  which 
most  of  the  constitutional  symptoms  in  the  form  of  leuco- 
derma,  alopecia,  and  general  enlargement  of  the  glands  are 
found.  The  eruption  disappears  without  leaving  any  scars, 
and  very  often  the  initial  lesion,  even  in  men,  leaves  no 
trace.  In  such  cases  the  Wassermann  reaction  will  often 
disclose  the  cause. 

The  success  of  the  therapy  cannot  always  be  regarded 
as  a  determining  factor  in  the  diagnosis,  because  we  know 
that  hemiplegias  which  are  not  of  specific  origin  may  im- 
prove or  even  entirely  disappear.  Our  chief  dependence 
in  the  diagnosis  must  be  on  the  proof  of  a  previous  syphilis 
and  the  exclusion  of  all  other  etiological  factors. 

Differential  Diagnosis  Apoplexy. — In  the  consideration  of 
the  differential  diagnosis  one  should  mention  that  occa- 


NERVOUS  LUES  AND  SPECIFIC  ENDARTERITIS      73 

sional  cases  of  apoplexy  have  been  reported  in  compara- 
tively young  persons,  where  the  autopsy  revealed  no 
apparent  cause. 

The  relapsing  type  of  hemiplegia  as  well  as  triplegia 
sometimes  occurs  in  individuals  who  have  not  yet  attained 
the  age  of  arteriosclerosis,  who  have  sound  internal  organs 
and  who  never  have  had  a  specific  infection. 

Brain  Tumor. — Tumor  of  the  brain  may  exhibit  for  a  long 
time  the  general  symptoms  of  headache,  insomnia,  and 
weakness  of  memory,  and  then  suddenly  either  with  or 
without  apoplectic  symptoms  develop  a  hemiplegia  without 
any  discoverable  increase  in  the  intracerebral  pressure. 

Brain  tumor  may  also  manifest  itself  as  an  acute  hemi- 
plegia with  scarcely  noticeable  or  with  no  prodromal  symp- 
toms whatever. 

Multiple  Sclerosis. — Transient  paralyses  can  occur  in 
multiple  sclerosis.  This  usually  occurs  in  young  persons 
with  healthy  internal  organs,  and  may  attack  individuals 
who  have  had  a  previous  specific  infection.  Although  the 
further  course  of  the  disease  as  a  rule  renders  the  situa- 
tion clear,  one  must  not  forget  on  the  other  hand  that  multi- 
ple sclerosis  not  infrequently  shows  very  marked  improve- 
ment, and  because  of  this  gives  further  support  to  the 
assumption  of  a  specific  hemiplegia. 

The  four  reactions  are  especially  valuable  in  differential 
diagnosis  here. 

Bright's  Disease. — Bright 's  disease  in  the  form  of  ursemic 
hemiplegia  may  also  present  difficulties  in  differential  diag- 
nosis. I  saw  a  case  in  which  there  existed  the  combination 
of  a  former  brain  syphilis  and  Bright's  disease.  The  ^epi- 
leptic convulsions  which  the  patient  developed  I  regarded 
as  caused  by  the  syphilis.  The  examination  did  not  give 
any  positive  evidence  of  Bright's  disease.  The  autopsy, 
however,  proved  that  the  convulsions  were  due  to  the 
nephritis  instead  of  the  brain  syphilis. 

Hysteria. — Hysterical  hemiplegias  in  syphilitic  patients 
may  also  cause  mistaken  diagnoses. 

Severe  attacks  of  migraine  may  not  only  cause  tran- 
sient motor  aphasia  and  hemianopsia,  but  also  produce 


74  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

hemiparesis.  If  a  person  with  such,  attacks  has  also  lues, 
a  differentiation  as  to  the  nature  at  the  time  is  not  possible, 
and  a  further  observation  of  their  course  will  be  required. 
Apoplectiform  attacks  occur  in  dementia  paralytica  and 
may  exhibit  symptoms  of  monoplegia,  hemiplegia,  and 
isolated  aphasia. 

General  Paresis. — When  dementia  paralytica  presents 
only  general  symptoms,  such  as  dizziness,  loss  of  memory, 
and  decreased  capacity  for  work,  as  it  not  infrequently 
does,  or  as  also  happens  when  an  apoplectiform  attack 
occurs  before  the  development  of  somatic  symptoms,  such 
as  changes  in  the  form,  size,  and  function  of  the  pupils,  and 
disturbances  of  speech,  one  is  justified  in  the  assumption 
that  the  cause  is  due  to  the  arteritic  form  of  brain  syphilis. 
In  the  great  majority  of  these  cases  the  further  course  will 
soon  enable  one  to  distinguish  between  the  two  conditions. 

Tuberculosis. — Tuberculosis  may  also  lead  to  confusion 
in  the  diagnosis.  A  solitary  tubercle,  which  develops  in 
the  pons  (a  favorite  location  for  solitary  tubercle),  can 
for  a  long  time  simulate  a  slow-forming  basilar  thrombus. 
If  the  patient  happens  to  have  had  a  previous  infection  of 
syphilis  the  assumption  of  a  specific  arteritis  of  the  basilar 
artery  is  naturally  first  considered. 

When  a  patient  has  attained  the  age  of  arteriosclerosis 
a  definite  distinction  cannot  be  made  between  a  specific 
arteritis  and  one  due  to  arteriosclerosis.  Existing  symp- 
toms of  a  florid  syphilis  when  present  will  help  to  clarify 
the  diagnosis. 

Heart  Disease  in  Syphilitics. — Luetics  may  through  some 
other  disease  contract  a  heart  affection.  In  such  cases  it 
is  sometimes  an  open  question  which  one  of  the  two  diseases 
is  responsible  for  the  symptoms  of  paresis. 

Arteriosclerosis  in  Young  Syphilitics. — We  must  also  take 
into  consideration  that  we  sometimes  find  an  arterioscle- 
rosis in  comparatively  young  individuals  which  has  been 
developed  by  syphilis,  but  is  in  itself  non-specific  in  charac- 
ter, and  that  this  condition  may  be  the  cause  of  the  existing 
brain  disease. 


NERVOUS  LUES  AND  SPECIFIC  ENDARTERITIS     75 

Summary. — The  most  important  factors  in  the  differ- 
ential diagnosis  must  be  continually  kept  in  mind.  If  a 
paralysis  occurs  in  a  young  person  or  a  person  of  middle 
age  in  whom  both  the  central  and  peripheral  arterial  system 
is  normal,  and  in  whom  there  exists  no  other  demonstrable 
cause  for  the  formation  of  embolic,  thrombotic  and  apoplec- 
tic processes,  in  whom  syphilis  has  either  been  proven  or 
seems  probable,  and  in  whom  transient  paralyses  in  the 
form  of  either  mono-,  hemi-,  or  triplegia  occur,  which  either 
entirely  disappear  or  show  marked  improvement,  and  which 
not  infrequently  leave  the  qualities  of  the  mind  unimpaired, 
we  are  justified  in  regarding  a  specific  arteritis  as  the  causa- 
tive factor.  In  all  such  cases  the  application  of  the  four 
reactions  and  their  findings  should  never  be  neglected  in 
making  a  differential  diagnosis, 


IV 

SYPHILITIC  CEREBRAL  MENINGITIS 

THE  membranes  of  the  brain  seem  particularly  sus- 
ceptible to  syphilis.  If  Wunderlich  should  state  at  the  pres- 
ent time  that  there  were  no  characteristic  symptoms  of 
brain  syphilis,  in  the  light  of  the  rich  experience  of  the  last 
25  years,  we  should  feel  called  upon  to  place  a  certain 
degree  of  limitation  upon  his  statement.  It  was  Virchow 
who  first  called  our  attention  to  the  juxtaposition  of  tumor 
formation  and  regression  in  syphilitic  tissue.  He  saw,  on 
the  one  hand,  in  the  reabsorption  and  shrinking  of  the  nerve 
compressing  gumma,  and,  on  the  other,  in  the  increase  of 
diseased  processes  through  recent  destruction  and  incom- 
plete absorption,  the  explanation  of  the  rapid  changes  in 
the  clinical  symptoms  which  frequently  occur  in  syphilis  of 
the  central  nervous  system.  Heubner  had  already  called 
attention  to  the  fact  that  in  many  cases  the  appearance  and 
disappearance  of  symptoms  of  paralysis  and  irritation  dom- 
inated the  clinical  picture.  Oppenheim  has  expressed  him- 
self in  this  regard  very  distinctly.  He  says:  "We  know  of 
no  other  neoplasm  in  which  both  of  these  processes,  growth 
and  retrograde  metamorphosis,  in  a  certain  sense  go  hand 
in  hand  and  are  so  closely  related,  so  that  the  scarcely 
formed  neoplasm  is  destroyed  and  upon  the  site  of  its 
location  another  immediately  develops." 

Incompleteness  and  Transient  Character  of  the  Clinical 
Symptoms. — When  we  combine  the  changes  in  the  lumina 
of  the  large  vessels  on  the  base  of  the  brain,  and  the  con- 
ditions which  are  produced  in  the  interior  of  the  brain 
through  these  changes,  as  well  as  the  qualities  of  the  con- 
stant change  in  the  affected  tissue,  we  are  better  able  to 
understand  the  clinical  peculiarities  which  brain  lues 
exhibits.  We  can  also  understand  why  it  is  just  this  class 
of  cases,  as  Naunyn  has  statistically  proven,  which  are  the 
most  favorably  influenced  by  antispecific  therapy,  We  ob- 

76 


SYPHILITIC  CEREBRAL  MENINGITIS  77 

serve  in  these  cases  when  treated  with  mercury  and  iodid 
rapid  improvement  and  recoveries,  which  we  do  not  see  in 
the  non-specific  syphilitic  processes  in  the  arteries. 

Multiplicity  of  Symptoms. — Another  factor  which  is  rela- 
tively characteristic  of  brain  syphilis  is  the  multiplicity 
of  symptoms.  Specific  basilar  meningitis  can  not  only 
through  pressure  cause  arteritic  symptoms,  such  as  we 
have  discussed  in  the  last  chapter,  but  also  cause  the 
involvement  of  the  cranial  nerves,  either  collectively  or 
singly,  or  in  various  combinations,  unilateral,  bilateral, 
or  alternating. 

The  syphilitic  infiltration  on  the  base  may  only  involve 
the  meninges,  but  can  involve  the  brain  tissue  itself  as  well. 

The  nerve  tissue,  as  we  have  seen,  may  become  affected 
through  contiguity,  and  the  condition  of  encephalitis  gum- 
mosa  then  exists,  or  it  becomes  compressed  through  the 
uniform  infiltration  of  gummatous  masses  or  isolated 
nodules,  or  through  disease  of  the  arteries  in  the  meninges, 
thrombosis  and  obliteration  of  the  lumina  are  produced  and 
the  consequent  secondary  softening  and  necrosis  of  the 
nerve  substance  then  follows. 

We  should  remember  that  the  arteries  which  supply  the 
cortex  of  the  brain  and  the  medullary  tissue  beneath  are 
first  found  in  the  pia,  where  they  give  off  branches  before 
entering  the  brain  substance. 

The  etiology  of  specific  cerebral  meningitis  does  not 
differ  from  that  of  syphilitic  arteritis.  What  has  been  said 
about  the  latter,  in  regard  to  heredity,  the  probable  influ- 
ence of  the  intensity  and  type  of  the  infection,  the  age  of  the 
patient,  the  form  of  therapy,  the  later  exacerbations,  also 
with  reference  to  trauma,  alcohol  and  other  injurious  fac- 
tors, will  apply  equally  as  well  to  the  former. 

For  practical  reasons  we  will  consider  the  meningitis  of 
the  convexity  and  base  of  the  brain  separately.  The  differ- 
ence in  the  localization  of  the  inflammation  causes  a  dif- 
ference in  the  symptoms.  However,  it  cannot  be  definitely 
determined  in  many  cases  whether  certain  symptoms  are 
caused  by  disease  of  the  convexity  or  base,  and  whether 
or  not  a  combination  of  both  forms  does  not  exist,  as  the 


78  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

pathological  examination  in  the  great  majority  of  cases 
shows. 

Symptoms  of  Specific  Meningitis  of  the  Convexity. — Not  in- 
frequently meningitis  of  the  convexity  is  ushered  in  with 
general  symptoms ;  local  symptoms  may  be  lacking,  because 
there  are  no  large  arteries  in  this  region,  the  collateral  cir- 
culation is  exceedingly  rich  and  none  of  the  cranial  nerves 
originate  there.  On  the  other  hand,  disease  of  the  con- 
vexity can  cause  certain  characteristic  symptoms  which  per- 
mit of  a  localizing  diagnosis.  We  may  find  the  appearance 
here  of  symptoms  of  irritation  and  paralysis,  which  are 
caused  by  an  involvement  of  the  motor  cortical  centres, 
such  as  monoconvulsions  and  monoparesis. 

Headache. — The  most  frequent  and  almost  never-failing 
general  symptom  is  the  headache.  It  may,  for  a  long  time, 
precede  all  other  symptoms,  may  be  localized  to  one  spot  or 
diffuse  in  nature,  dull  or  severe,  and  not  infrequently  the 
patients  are  stupefied  by  it.  Frequently — however,  not  so 
frequently  in  my  experience  as  Fournier  describes  and  as 
the  text-books  since  then  would  have  us  believe — the  pain 
becomes  worse  during  the  night.  The  headache  depends 
on  an  affection  of  the  dura,  for  there  are  no  pain-supplying 
elements  either  in  the  arachnoid  and  pia  or  in  the  brain- 
substance  itself.  Sometimes  one  finds  in  such  cases  a  local 
tenderness  to  percussion,  another  symptom  for  the  assump- 
tion of  superficial  disease  of  the  brain.  The  headache  may 
appear  soon  after  the  infection,  or  years  may  pass  before 
it  makes  its  appearance.  The  following  cases  will  illustrate 
this : 

A  coachman,  forty-five  years  old,  twenty  years  after  a 
syphilitic  infection,  became  affected  with  severe  headache 
and  at  the  same  time  dizziness.  The  headache  was  so  severe 
that  the  patient  sat  around  in  an  apathetic  manner,  and  at 
times,  for  short  periods,  was  unconscious.  There  was  no 
vomiting  and  no  convulsions;  objectively  the  internal 
organs  and  nervous  system  were  normal.  Under  a  three 
weeks'  treatment,  with  large  doses  of  potassium  iodid,  the 
pain  entirely  disappeared.  Two  years  later  the  patient 
suffered  a  relapse,  and  again  presented  the  same  severe 


SYPHILITIC  CEREBRAL  MENINGITIS  79 

stupefying  headache,  which  this  time,  under  an  energetic 
mixed  treatment,  disappeared  in  two  weeks. 

The  next  case  developed  a  specific  headache  one  year 
after  the  infection,  at  the  same  time  exhibiting  secondary 
symptoms  in  the  skin  and  mucous  membrane. 

A  married  woman,  twenty  years  old,  complained  for  six 
weeks  of  an  exceedingly  severe  headachy,  which  tortured  her 
day  and  night,  so  that  she  was  incapable  of  thinking  or  sleep- 
ing. At  the  same  time  she  had  nausea  without  vomiting. 
It  was  ascertained  that  her  husband  had  contracted  syphilis 
a  year  before  his  marriage,  and  that  he  had  taken  treatment 
for  secondary  symptoms  within  the  last  six  months.  Physi- 
cal examination  of  the  young  woman  showed  secondary 
symptoms,  both  in  the  skin  and  mucous  membrane.  The 
objective  symptoms  on  the  part  of  the  nervous  system 
were  diffuse  sensitiveness  of  the  head  to  percussion.  All 
movements  of  the  head  were  painful,  the  papillae  of  both 
optic  nerves  hyperaemic,  tendon  reflexes  of  both  upper  and 
lower  extremities  very  active,  and  sensations  of  dizziness, 
and  swaying  in  standing  and  walking.  With  ten  inunctions 
of  6.0  each  of  unguentum  ciner.  all  the  symptoms  disap- 
peared entirely. 

It  is  not  at  all  infrequent  that  a  syphilitic  headache 
occurs  early  and  also  late,  monosymptomatic  after  an  infec- 
tion, so  that  in  every  case  of  severe  and  stubborn  headache 
one  should  at  least  think  of  syphilis.  One  very  often  finds 
cases  in  which  along  with  the  headache  the  only  symptom  is 
an  anomaly  of  the  pupil,  of  which  the  following  case  is  an 
example : 

A  woman,  thirty-seven  years  old,  who  had  been  infected 
with  syphilis  twelve  years  before,  was  taken  sick  with  an 
extremely  severe  headache  and  occasional  vomiting.  Ob- 
jectively, in  addition  to  a  diffuse  sensitiveness  of  the  skull 
to  percussion,  there  was  anisocoria  (right  >  left)  and 
sluggishness  in  contraction  of  both  pupils.  Antispecific 
treatment  of  four  weeks '  duration  cured  the  headache  com- 
pletely. The  pupil  reaction  on  both  sides  became  normal, 
but  the  anisocoria  remained. 

Psychic    Anomalies. — The  psychic   anomalies   are   quite 


80  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

characteristic.  Attention  has  been  called  to  the  fact  that 
the  headache  is  often  so  severe  and  continuous  that  it  pro- 
duces disturbances  of  consciousness,  such  as  apathy  and 
general  dulness.  The  dream-  and  intoxicated-like  state 
which  was  described  in  connection  with  syphilitic  arteritis 
may  also  be  observed  here,  apparently  as  a  result  of  the 
induration  and  gummatous  infiltration  and  arterial  changes. 

Foufnier  has  described  a  severe  delirium  such  as  one 
sees  in  typhoid  or  encephalitis  and  acute  meningitis.  Cornil 
reported  a  case  of  an  eighteen-year-old  youth  with  a  severe 
delirium,  resembling  the  delirium  of  an  infection,  which 
was  ushered  in  by  general  convulsions  and  in  three  days 
terminated  with  a  fatal  result.  The  autopsy  revealed  a 
pachymeningitis  gummosa. 

In  many  cases  of  chronic  specific  meningitis  of  the  con- 
vexity the  chief  symptom  is  a  progressive  dementia.  The 
condition  begins  with  apathy  and  lack  of  interest ;  the  mem- 
ory becomes  poor.  Fournier  describes  an  "  amnesic  syph- 
ilitique" — the  powers  of  comprehension  are  weakened  and, 
without  either  exaltation  or  depression,  a  severe  dementia 
develops.  Other  symptoms  may  be  entirely  lacking,  except 
those  corresponding  to  the  general  decrease  in  the  personal 
well-being,  and  a  slowness  and  difficulty  in  the  movements 
because  of  this.  They  become  awkward  and  clumsy,  but  not 
paretic  and  ataxic.  The  gait  likewise  becomes  heavy,  the 
impulse  for  lifting  up  the  limbs  is  lacking,  the  ability  to 
maintain  the  equilibrium  suffers  because  of  a  lack  of  atten- 
tion and  the  patient  may  sway  and  totter.  In  this  stage 
one  not  infrequently  sees  the  clinical  picture  which  brain- 
tumor  sometimes  presents  as  the  only  expression  of  the 
severe  intracranial  disease. 

This  severe  clinical  picture  may,  under  suitable  treat- 
ment, disappear. 

A  Case  of  Slow-developing  Progressive  Dementia,  without 
Somatic  Symptoms,  Cured  by  Antispecific  Therapy. — A  banker, 
forty-six  years  old,  sixteen  years  before  his  present  illness 
had  contracted  syphilis.  For  about  six  months  he  found 
difficulty  in  doing  his  work.  He  also  lost  his  zest  for  amuse- 
ment. He  began  to  sleep  during  the  day,  and  at  night  slept 


SYPHILITIC  CEREBRAL  MENINGITIS  81 

as  if  he  were  dead.  He  lost  interest  in  his  business  and 
family.  He  did  not  observe  the  failure  in  his  mentality,  and 
was  astonished  because  his  family  thought  he  was  sick. 
Occasionally  he  complained  of  pains  in  his  head  and  of  dizzi- 
ness. He  became  gluttonous,  untidy  in  his  eating  and  dress, 
but  at  the  same  time  was  good-natured  and  peaceable.  He 
at  no  time  manifested  any  indication  of  ideas  of  grandeur 
or  periods  of  excitement.  His  speech  became  bad.  He  left 
out  words,  spoke  as  one  to  whom  it  seemed  difficult  to  keep 
awake;  at  the  same  time  there  was  no  syllable  stumbling 
and  no  aphasic  disturbance.  His  gait  was  heavy. 

Objectively  there  was  no  arteriosclerosis  and  nothing  to 
indicate  an  increase  of  intracranial  pressure.  The  charac- 
teristic somatic  symptoms  of  paresis  were  absent.  After 
this  patient  had  received  antispecific  treatment  for  four 
weeks  he  began  to  improve.  Little  by  little  he  awakened, 
became  stronger  mentally,  and  more  active  physically.  He 
has  remained  perfectly  well  now  for  eight  years.  At  my 
suggestion  he  has  taken  a  mixed  treatment  two  months  out 
of  each  year  for  four  years. 

Pupil  Anomalies. — The  pupil  anomalies  are  an  exceed- 
ingly important  and  frequent  symptom.  They  occur  not 
only  as  the  result  of  an  involvement  of  the  oculomotor 
nerve-trunk  and  its  nucleus,  but  also  may  occur  from  disease 
of  the  cortex. 

Recently  a  series  of  observations  concerning  the  changes 
in  the  size  of  the  pupil  in  hysteria  have  made  a  cortical 
localization  of  the  movements  of  the  pupil  probable.  Bech- 
terew  and  Piltz  have  demonstrated  experimentally  that 
from  certain  areas  of  the  cortex  pupillary  changes  can  be 
produced.  According  to  our  present  experience,  apart 
from  an  abnormal  reflex  activity,  we  are  not  justified  in 
considering  anomalies  of  the  pupil  as  possible  in  neuras- 
thenia. 

The  cases  of  so-called  neurasthenia  after  lues  are,  ex- 
cluding chronic  alcoholism,  if  pupil  anomalies  exist,  either 
in  the  category  of  dementia  paralytica  incipiens,  or  they 
are  the  clinical  expression  of  a  specific  meningitis,  either  of 
the  convexity  or  base  of  the  brain. 


82  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

The  pupillary  changes  of  general  paresis  do  not  respond 
to  antispecific  therapy. 

The  importance  of  the  behavior  of  the  pupils  in  a  differ- 
ential diagnosis  of  general  paresis  and  specific  meningitis 
of  the  convexity  is  illustrated  in  the  following  case : 

A  merchant,  thirty-five  years  old,  became  infected  with, 
syphilis  nine  years  ago.  He  took  one  short  course  of  inunc- 
tions at  the  time,  another  course  three  years  ago,  and  two 
years  ago  a  course  of  potassium  iodid.  The  patient  has 
shown  no  symptoms  of  syphilis  since  he  finished  his  inunc- 
tion treatment.  He  has  never  been  a  potator.  His  disposi- 
tion has  changed  in  the  last  six  months.  He  becomes 
irritated  very  easily,  is  careless  of  his  personal  appearance, 
and  is  forgetful.  During  the  past  few  weeks  he  forgets 
to  urinate,  loses  his  way  on  the  street,  and  wants  to  sleep 
all  the  time.  He  complains  occasionally  of  headache  and 
dizziness.  Objectively  no  demonstrable  evidences  of  syph- 
ilis were  discovered.  His  condition  indicated  apathy  and 
dementia.  There  existed  severe  disturbances  of  his  intelli- 
gence, memory,  and  power  of  orientation. 

There  was  no  paralysis  of  the  cranial  nerves  and  no 
speech  disturbances,  except  that  his  speech  corresponding 
to  his  dementia  was  toneless  and  indistinct.  There  were 
no  spinal  symptoms  and  the  patellar  reflexes  were  lively. 

The  pupils  were  of  medium  size  and  equal ;  reaction  both 
to  light  and  convergence  was  sluggish.  Otherwise  the  eyes 
were  normal. 

A  course  of  inunctions  was  begun  immediately.  In  four 
weeks  the  change  in  the  patient  was  marvellous.  His  normal 
interest  and  activity  returned.  He  became  careful  of  his 
personal  appearance,  clean  in  his  habits,  could  do  small 
examples  without  mistake,  wrote  both  the  history  of  his 
life  and  the  history  of  his  sickness  without  errors  in  spelling 
or  composition,  and  was  free  from  headache  and  dizziness. 
By  more  careful  examination,  however,  numerous  defects 
in  his  intelligence  and  memory  became  apparent. 

The  pupil  anomalies  remained  exactly  the  same  and 
the  patellar  reflexes  were  still  lively. 

The  diagnosis  "remission  in  a  case  of  general  paresis" 


83 

was  made  because  of  the  behavior  of  the  pupils.  After 
remaining  apparently  well  for  a  year,  he  quickly  developed 
the  mental  picture  of  dementia  paralytica. 

Combination  of  Paresis  with  Specific  Meningitis  of  the 
Convexity. — Meningitis  of  the  convexity  may  also  exist  in 
combination  with  general  paresis,  with  the  patient  manifest- 
ing symptoms  of  both  affections. 

A  woman,  thirty-one  years  old,  who  had  been  treated 
eight  years  before  for  secondary  syphilis,  became  unable 
to  work  because  of  severe  attacks  of  headache  preceded  by 
dizziness,  and  also  a  left-sided  hemiparesis,  which  had  in 
the  main  receded  but  had  left  behind  a  condition  of  physical 
weakness.  She  had  not  been  addicted  to  the  use  of  alcohol. 
She  was  received  into  the  hospital  because  of  attacks  of 
unconsciousness. 

At  the  time  of  her  entrance  she  was  in  a  stupor ous  con- 
dition, showed  symptoms  of  motor  aphasia  and  agraphia, 
slight  paresis  of  the  right  side.  The  pupils  reacted  some- 
what sluggishly,  there  was  no  hemianopsia,  the  eye  back- 
grounds were  normal,  all  the  tendon  reflexes  were  lively, 
right  more  active  than  left.  The  cranium,  in  the  left  parie- 
tal region,  was  sensitive  to  percussion. 

Under  a  mixed  treatment  the  stupor  gradually  disap- 
peared and  a  moderate  degree  of  dementia  took  its  place. 
The  patient  gradually  became  brighter;  in  speaking  a 
paraphasic  defect  was  often  manifest.  Her  severe  attacks 
of  headache  returned  and  she  developed  ideas  of  grandeur. 
The  dementia  gradually  deepened,  and  the  epileptic  attacks 
began  again,  in  one  of  which  she  died. 

The  autopsy  revealed  the  dura  mater  adherent  to  the 
skull,  and  over  the  frontal  region  much  thickened.  The  pia 
over  the  parietal  and  temporal  regions  was  cloudy  and 
thickened,  adherent  in  places  to  the  cortex,  showing,  in  part, 
gummatous  infiltrations  which  had  extended  to  the  cortex. 
The  ependyma  of  the  enlarged  ventricles  was  thickened 
and  granulated.  The  arteries  of  the  base  and  the  arteria 
fossae  sylvii  and  arteria  profundse  were  not  altered. 

The  microscopic  examination  of  the  cortex  demonstrated 
the  following  changes : 


84  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

First.  Marked  disappearance  of  the  tangential  fibres, 
thickening  and  increase  of  the  glia. 

Second.  Degeneration  of  varying  degrees  of  the  gan- 
glion cells. 

Third.  Thickening,  and  in  part  hyaline  degeneration, 
of  the  arterioles  of  the  cortex. 

In  this  case  the  pathology  justifies  one  in  regarding 
the  tenderness  to  percussion,  the  attacks  of  epilepsy,  the 
stuporous  condition,  and  probably  the  hemiparesis  and 
aphasia  as  caused  by  the  pachy-  and  leptomeningitis,  and 
to  assume  that  their  partial  disappearance  was  due  to  the 
antispecific  therapy.  As  a  matter  of  fact,  fresh  infiltrations 
of  the  meninges  existed  alongside  of  old  cicatrized  thicken- 
ings. The  psychic  changes  should  be  attributed  to  the  de- 
mentia paralytica,  whose  pathology  was  demonstrated  in 
the  changes  of  the  ganglion  cells,  the  disappearance  of  the 
tangential  fibres,  and  the  proliferation  of  the  glia. 

Choked  Discs. — Choked  discs  should  also  be  enumerated 
under  the  general  symptoms.  This  condition  is  more  apt  to 
occur  in  a  basilar  meningitis,  but  may  appear  occasionally 
in  a  cortical  encephalitis  gummosa. 

Cortical  Convulsions. — Under  cortical  convulsions,  we 
understand,  of  course,  according  to  Jackson's  description, 
a  discharge  of  muscular  contractions,  tonic  convulsions  by 
retained  consciousness,  or  with  the  consciousness  only 
slightly  disturbed.  The  contractions  may  be  confined  either 
to  one  extremity,  or  only  a  part  of  the  same  (as  the  finger 
or  hand),  or  they  may  extend,  according  to  the  discharge 
of  the  excitant,  involving  one  centre  after  another,  until 
the  upper  and  lower  extremities  and  the  face  and  tongue 
become  involved. 

One  can  only  be  positive  in  diagnosing  the  source  of  the 
convulsion,  as  existing  in  the  cortex,  when  paralyzed  or 
parietic  parts  become  involved  in  the  jerkings  or  spasms. 
Sometimes,  however,  the  accompanying  symptoms  will  per- 
mit of  the  establishing  of  a  cortical  origin  for  the  convul- 
sions without  the  necessity  of  there  first  being  a  paralysis. 

If  the  syphilitic  changes  in  the  meninges  are  limited, 


SYPHILITIC  CEREBRAL  MENINGITIS  85 

convulsions  will  be  the  result  when  these  changes  occur  over 
the  motor  area.  When  the  involvement  of  the  meninges  is 
extensive  we  not  infrequently  see  the  patients  affected  with 
general  epileptic  attacks,  which  cannot  be  distinguished 
from  the  attacks  of  idiopathic  epilepsy,  if  in  the  periods 
between  the  attacks,  headaches,  slight  defects  in  memory, 
and  an  insignificant  dulling  of  the  mental  powers  are  not 
discovered  by  careful  examination,  or  if  intercurrent  motor 
symptoms  of  irritation — such  as  tremor,  isolated  muscle 
contractions,  or  even  permanent  conditions  of  contracture — 
are  not  present. 

From  our  experience  with  brain  tumors  we  know  that 
from  almost  any  spot  in  the  brain  general  convulsions  may 
be  liberated,  so  we  should  not  be  astonished  if  a  gumma 
located  in  the  substance  of  the  brain  causes  an  epilepsy, 
which  does  not,  however,  bear  the  character  of  a  cortical 
epilepsy. 

Hemiepilepsy. — The  hemiepilepsy,  even  in  a  severe  form, 
does  not  always  present  a  macroscopic  or  a  microscopic 
recognizable  pathological  basis,  as  Leo  Miiller  has  shown 
from  cases  in  my  department  at  Eppendorf. 

Heubner  has  called  attention  to  the  frequency  of  epilep- 
tic convulsions.  He  observed  in  45  cases  of  gummatous 
meningitis  of  the  brain  convulsions  in  26  cases. 

The  views  concerning  the  frequency  of  hemiepilepsy 
differ.  We  find  the  greatest  variation  when  we  compare 
Charcot's  view,  who  regarded  hemiepilepsy  as  of  frequent 
occurrence  in  syphilis,  with  Naunyn's  experience,  who 
found,  out  of  105  cases,  only  a  single  case  of  hemiepilepsy. 

I  cannot  regard  the  occurrence  of  hemiepilepsy  so  rare 
as  does  Naunyn.  I  have  seen  this  form  of  epilepsy  numer- 
ous times,  both  monosymptomatic  and  in  combination  with 
other  symptoms. 

It  may  happen  that  the  hemiepileptic  attacks  appear  as 
the  first  symptom  of  the  disease.  However,  this  is  not  fre- 
quent, much  less  frequent  than  in  brain  tumor.  Almost 
always  a  more  or  less  severe  headache  and  general  symp- 
toms precede  the  epilepsy. 


86  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

The  cortical  convulsions  can  present  every  shade  of 
transition,  from  a  typical  Jacksonian  epilepsy  to  general 
epileptic  convulsions. 

One  should  remember  that  in  brain  syphilis  the  convul- 
sions represent  only  a  part  of  a  long  chain  of  brain 
symptoms. 

The  cortical  paralyses  may  appear  as  monopareses  or 
monoparalyses,  or  also  as  hemipareses  or  hemiparalyses. 
These  paralyses  may  develop  either  slowly  or  in  an  acute 
manner.  The  acuteness  or  chronicity  of  development  is  de- 
termined by  the  pathological  conditions. 

The  cortical  paralyses  may  precede  the  convulsions,  may 
follow  them,  or  may  appear  without  any  convulsive  symp- 
toms. Cases  of  isolated  cortical  motor  symptoms,  however, 
are  rare.  In  the  great  majority  of  cases,  along  with  the 
cortical  symptoms  of  convulsions  and  paralysis,  are  found 
basilar  meningeal  and  arteritic  symptoms. 

The  following  case  is  an  example  of  a  pure  cortical  epi- 
lepsy without  any  objective  somatic  symptoms: 

A  man,  thirty-three  years  of  age,  became  infected  with 
syphilis  in  1891.  He  had  been  healthy  before,  and  his  family 
history  was  good.  The  initial  lesion  and  secondary  symp- 
toms were  thoroughly  treated.  On  his  return  from  a  trip 
to  New  York,  after  he  had  complained  of  headache  and 
dizziness  several  days  before,  he  was  suddenly  seized  with 
epileptic  convulsions,  which  were  localized  on  the  left  side 
and  accompanied  by  only  slight  disturbances  of  conscious- 
ness. His  objective  examination  revealed,  as  the  only  evi- 
dence of  a  previous  lues,  a  slight  indolent  swelling  of  the 
glands  of  the  neck  and  inguinal  region.  He  complained 
of  a  dull  headache  and  at  times  of  dizziness,  also  of  sensa- 
tions of  numbness  and  crawling  in  his  left  leg,  which  he 
did  not  feel  sure  of  in  walking. 

The  cranial  nerves,  pupils,  and  eye  backgrounds  were 
normal.  An  abnormal  sensitiveness  to  percussion  was  pres- 
ent on  the  right  anterior  and  middle  part  of  the  cranium. 
Sensation  and  both  skin  and  tendon  reflexes  were  normal. 

Under  inunctions  and  the  internal  administration  of  po- 
tassium iodid,  the  headache  and  dizziness  improved.  A  week 


87 

after  beginning  treatment,  without  any  warning,  while  the 
patient  sat  at  the  table,  he  was  seized  with  another  epileptic 
attack,  in  which  he  became  unconscious,  with  convulsive 
movements  on  the  left  side  of  the  body.  Three  days  after 
this  he  had  still  another  attack,  where  the  convulsions  were 
limited  to  the  left  side,  but  in  which  he  did  not  lose  con- 
sciousness. This  was  his  last  seizure.  Three  months  later 
he  returned  to  New  York,  apparently  well. 

The  following  case  is  also  one  of  cortical  epilepsy  with 
choked  discs : 

A  plumber,  thirty  years  old,  had  contracted  lues  six 
years  before.  He  had  taken  no  treatment  for  it.  For  four 
weeks  he  had  noticed  sensations  of  creeping  and  a  furry 
feeling  on  the  left  side  of  his  body.  One  day,  without  any 
apparent  cause,  his  left  leg  was  suddenly  drawn  up  with  a 
cramp.  The  next  day  this  occurred  again,  and  the  patient 
lost  consciousness  and  fell  to  the  ground.  His  landlady 
stated  that  during  his  unconsciousness  he  had  muscular 
twitchings  in  his  left  side.  For  about  five  minutes  after  his 
attack  he  complained  of  headache,  but  went  to  work.  The 
following  day  he  had  such  a  severe  headache  that  he  was 
compelled  to  quit  work.  A  few  days  later  he  was  received 
into  the  hospital,  having  had  repeated  convulsive  attacks 
in  his  left  side.  The  patient  in  walking  dragged  his  left 
leg  a  little.  The  tendon  reflexes  on  the  left  side  were  in- 
creased, the  skin  reflexes  decreased.  The  skull  above  the 
right  central  convolutions  was  sensitive,  both  to  pressure 
and  percussion.  In  the  background  of  the  eyes  there  was  a 
well-marked  choked  disc.  The  pulse  was  slow,  between  50 
and  60. 

Under  a  mixed  treatment  all  the  symptoms  gradually 
disappeared.  The  choked  discs  remained  the  longest. 

A  Case  of  Hemiparesis  Apoplectica  Arteritica. — A  woman, 
forty-eight  years  old,  was  infected  by  her  husband  twenty 
years  before.  She  took  at  the  time  a  course  of  inunctions, 
later  aborted  several  times,  gave  birth  to  two  dead  children, 
and  then  two  living  ones,  who  soon  died.  Otherwise  she  had 
always  been  healthy.  For  four  months  she  had  complained 
of  severe  headaches,  and  paraesthesias  in  the  right  upper 


88  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

and  lower  extremities,  of  dizziness  coming  on  in  attacks, 
and  spells  of  an  uncontrollable  desire  to  sleep,  alternating 
with  insomnia.  Three  days  before  her  entrance  into  the 
hospital  she  fell  suddenly  to  the  ground  and  became  para- 
lyzed in  her  right  side. 

Physical  examination  revealed  a  right-sided  paralysis 
of  the  extremities,  with  paresis  of  the  right  facial,  and  slight 
disturbances  of  consciousness.  There  were  no  signs  of 
intracranial  pressure.  There  was  present  in  both  eyes  a 
chorioretinitis,  which  from  the  ophthalmologic  side  was 
regarded  as  specific.  Antispecific  treatment  was  begun  im- 
mediately. Three  days  after  her  reception  into  the  hospital 
the  patient  was  seized  with  convulsions  on  the  right  side  and 
became  unconscious.  These  attacks  were  repeated  in  the 
course  of  the  next  two  weeks  several  times  and  extended  to 
the  left  side  of  the  body.  These  finally  gradually  disap- 
peared, and  after  three  months'  treatment  the  patient  wa,s 
discharged.  A  slight  paresis  still  existed  in  the  right  upper 
extremity,  and  the  deep  reflexes  on  this  side  were  more 
active  than  on  the  other  side.  The  chorioretinitis  was 
greatly  improved,  and  the  headache  had  entirely  disap- 
peared. 

With  reference  to  the  etiological  role  of  trauma,  the  fol- 
lowing case  is  interesting: 

Meningitis  Convexitatis  Luetica  Post  -  traumatica.  —  A 
farmer,  fifty-five  years  old,  was  seen  by  me  for  the  first  time 
on  March  31,  1899.  Eleven  weeks  before  he  had  fallen  from 
a  hay-loft  to  the  floor  of  his  barn  and  had  struck  upon  his 
neck  and  the  left  side  of  his  head.  After  about  two  weeks, 
when  the  symptoms  of  a  spinal  meningeal  hemorrhage  had 
disappeared,  the  patient  seemed  fairly  well,  until  six  weeks 
later,  when  a  headache,  which  was  localized  on  the  left  side 
of  the  head,  made  its  appearance.  The  pains  increased  in 
severity.  The  patient  had  a  feeling  as  if  the  scalp  was 
too  tight,  had  a  constant  desire  to  sleep,  and  complained  of 
dizziness.  His  gait  was  unsteady.  He  developed  a  weak- 
ness in  his  right  arm,  so  that  he  was  unable  to  write;  also 
depression  and  attacks  of  fear  of  impending  death.  In  the 
last  two  weeks  he  experienced  involuntary  twitchings  in  the 


SYPHILITIC  CEREBRAL  MENINGITIS  89 

fingers  of  his  right  hand.  During  the  last  four  days  he  had 
two  convulsive  attacks  involving  the  whole  right  side,  be- 
ginning in  the  leg  and  extending  upwards. 

His  history  in  regard  to  lues  was  somewhat  indefinite. 
While  a  widower,  after  having  had  three  children  by  his 
first  wife,  he  developed  an  ulcer  of  doubtful  character,  for 
which  he  never  took  any  antispecific  treatment.  Afterwards 
he  married  again.  His  second  wife  had  four  successive 
miscarriages,  then,  after  a  course  of  treatment  with  mercury 
and  iodid,  gave  birth  to  a  child,  which  died  one  year  later, 
with  enlarged  glands.  While  she  was  carrying  the  next 
child  the  mother  took  potassium  iodid;  The  child  came  at 
full  term,  and  still  lives.  The  next  child  (during  the  preg- 
nancy the  mother  took  no  treatment)  died  two  weeks  after 
birth  with  marasmus. 

The  examination  showed  tenderness  of  the  left  side  of 
the  skull  to  percussion,  which  seemed  most  localized  over 
the  upper  and  middle  parts  of  the  central  convolutions.  The 
entire  right  side  was  weak.  The  powers  of  localization, 
position,  and  astereognosis  in  the  right  upper  extremity 
were  somewhat  lacking.  Otherwise  no  other  disturbances 
could  be  determined.  I  prescribed  a  thorough  treatment 
with  mercury  and  iodid,  upon  the  assumption  that,  as  the 
result  of  a  head  injury  in  an  individual  who  had  previously 
had  syphilis,  a  localized  specific  meningeal  infection  had 
developed. 

The  patient  took  daily,  for  six  weeks,  inunctions  contain- 
ing four  grammes  of  mercury  and  internally  three  grammes 
of  potassium  iodid.  During  the  first  fourteen  days  of  the 
treatment  the  patient  grew  worse  rather  than  better.  He 
was  much  depressed,  and  seldom  spoke,  and  then  it  was 
difficult  for  him  to  think  of  the  words  he  wished  to  use. 
After  fourteen  days  he  began  to  improve  slowly,  and  even- 
tually made  a  good  recovery. 

The  diagnosis  of  disease  of  the  meninges  may  be  deter- 
mined by  the  cortical  nature  of  the  convulsions  which  take 
place  in  the  paraesthetic  and  paretic  extremities,  by  the 
sensitiveness  to  percussion  over  the  areas  corresponding  to 
the  parts  affected,  and  by  the  character  of  the  sensory  dis- 


90  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

turbance.  The  specific  nature  of  the  disease  must,  of  course, 
be  elicited  from  the  previous  history  of  the  case,  the  ex- 
amination, and  the  effect  of  antispecific  therapy. 

General  Epileptic  Convulsions. — General  epileptic  convul- 
sions may  be  more  frequent  than  hemiepilepsy,  the  first 
precursors  of  other  cerebral  symptoms.  They  occur,  how- 
ever, almost  always  accompanied  with  the  general  symptoms 
of  headache,  dizziness,  and  vomiting.  Disturbances  of  and 
loss  of  consciousness  may  not  take  place,  although  con- 
sciousness is  usually  affected. 

We  know  that  specific  disease  of  the  cortex  can  produce 
both  hemiepilepsy  and  general  epilepsy,  but  it  has  further 
been  proven  that  through  syphilis  an  individual  can  become 
epileptic  without  the  development  of  specific  changes  in 
the  central  nervous  system.  In  other  words,  an  epilepsy  of 
the  character  of  the  genuine  (not  secondary  in  the  sense 
of  Nothnagel's)  under  the  influence  of  a  luetic  infection 
may  both  appear  and  remain  as  monosymptomatic  in  char- 
acter. This  condition  will  be  further  discussed,  and  cases 
cited,  in  the  chapter  on  "Neuroses  in  Syphilis." 

Sensory  Disturbances  of  a  Cortical  Origin. — Sensory  dis- 
turbances emanating  from  the  cortex  are  not  often  observed. 

The  following  is  an  interesting  case  of  sensory  hemi- 
epilepsy : 

A  merchant,  thirty-eight  years  old,  eight  years  before 
had  acquired  syphilis.  He  consulted  his  physician  because 
of  attacks  of  unilateral  paraethesias.  The  crawling  sensa- 
tion and  furry  feeling  began  in  the  face,  extended  to  the 
upper  and  then  the  lower  extremities.  These  paraesthesias 
were  regularly  accompanied  by  dizziness  and  a  partial  loss 
of  consciousness.  After  the  attack,  which  lasted  about  fif- 
teen minutes,  the  patient  complained  of  headadie  and  a 
general  feeling  of  weakness.  Symptoms  of  motor  irritation 
never  appeared. 

Cortical  Speech  Disturbances. — Cortical  disturbances  of 
the  speech  are  more  frequent.  The  motor  or  sensory 
aphasia  can,  of  course,  only  be  regarded  as  cortical  when 
cortical  symptoms  are  present. 

The  aphasia  is  often  only  slightly  indicated.    The  patient 


91 

has  difficulty  in  finding  the  right  expression,  or  makes  para- 
phasic  mistakes.  In  other  cases  the  aphasic  disturbance 
is  only  of  short  duration,  coming  in  an  attack  and  then 
permanently  disappearing,  or  reappearing  in  more  severe 
form.  When  the  aphasia  persists  it  is  usually  accompanied 
with  either  symptoms  of  motor  irritation  or  paralysis  in 
the  face  and  extremities. 

Hemianopsia. — Hemianopsia  can  also  appear  as  a  symp- 
tom of  cortical  meningeal  affection.  The  best  known  case 
is  the  one  of  Pooley,  although  in  this  case  the  right-sided 
hemianopsia  and  hemiparesis  were  not  entirely  derived 
from  the  cortex,  for  the  specific  growth  had  penetrated  into 
the  brain  substance  of  the  left  occipital  lobe. 

Differential  Diagnosis  of  Meningitis  Syphilitica  Cerebralis 
Corticalis.  Headache  from  Local  Causes. — Where  the  head- 
ache appears  as  an  isolated  symptom,  other  causes,  such  as 
chronic  ear  and  nasal  affections,  arteriosclerotic  changes  in 
the  arteries,  disease  of  the  kidneys,  and  tumor  or  abscess 
of  the  brain  of  non-luetic  origin  must  be  excluded. 

Simple  Headache. — One  should  also  remember  that  a 
luetic  may  be  affected  with  a  simple  headache.  In  the  dif- 
ferential diagnosis  of  such  cases  the  examination  of  the 
spinal  fluid  with  reference  to  the  pleocytosis,  globulin,  and 
Wassermann  reactions  will  usually  enable  one  to  determine 
the  difference. 

Headache  Due  to  Uraemia. — If,  in  a  syphilitic  patient  with 
headache,  albumin  is  found  in  the  urine,  it  may  be  difficult 
sometimes  to  decide  whether  the  brain  symptoms,  headache, 
vomiting,  epileptiform  convulsions,  and  stupor  are  the  ex- 
pression of  a  specific  brain  affection  or  of  a  uraBmia.  Ex- 
amination of  the  spinal  fluid  will  in  such  conditions  be 
extremely  valuable  in  differentiating  between  these  two 
conditions. 

Syphilis,  as  is  well  known,  does  not  cause  contracted 
kidneys.  We  see  the  effects  of  syphilis  on  the  kidneys  in 
the  form  of  a  circumscribed  interstitial  nephritis  with  and 
without  gummatous  formation,  as  well  as  amyloid  degener- 
ation, most  often  combined  with  diffuse  chronic  parenchy- 
matous  disease.  This  form  of  kidney  affection  is  particu- 


92  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

larly  frequent  in  combination  with  specific  disease  of  the 
liver. 

Headache  with  Pachymeningitis  Alcoholica. — In  other  cases 
a  complicating  alcoholism  will  make  it  difficult  to  decide 
whether  the  pachymeningitis  is  due  to  syphilis  or  alcohol. 
Pachymeningitis  alcoholica  may  appear  under  the  general 
symptoms  of  a  diffuse  or  localized  headache,  accompanied 
at  the  same  time  by  attacks  of  stupor  and  heavy  sleep,  also 
by  localized  convulsions,  by  hemiepileptic  and  general 
epileptic  attacks,  and  by  paresis  and  paralytic  conditions 
of  the  extremities. 

Tumor  Cerebri. — True  cortical  convulsions  do  not  always 
have  a  visible  or  palpable  pathological  basis,  which  natur- 
ally should  cause  one  to  be  rather  cautious  in  the  diagnostic 
utilization  of  cortical  epileptic  convulsions,  even  in  syph- 
ilitics.  On  the  other  hand,  we  know  that  disease  of  the  cor- 
tex may  appear  under  the  clinical  picture  of  general  epilep- 
tic convulsions,  while  tumors  and  softenings  situated  in 
other  portions  of  the  brain,  and  which,  except  reflexly,  have 
nothing  to  do  with  the  cortex,  can  cause  cortical  convulsions. 

Case  of  tumor  in  the  motor  centres  of  the  cortex  with 
general  convulsions : 

A  woman,  forty  years  old,  had  married  a  syphilitic  hus- 
band, who  later  died  of  brain  syphilis.  After  several  abor- 
tions she  gave  birth  to  normal  children.  Three  months  be- 
fore her  death  she  began  to  complain  of  headache.  Then 
one  day  she  was  suddenly  seized  with  general  epileptic  con- 
vulsions, severe  in  character,  followed  by  headache  and  vom- 
iting. In  addition  to  these  symptoms  she  had  attacks  of 
disturbed  consciousness,  alternating  with  complete  clear- 
ness. The  patient  died  in  a  severe  epileptic  attack. 

Physical  examination  four  days  before  death  revealed  a 
slight  motor  weakness  of  the  right  side  of  the  body  with  in- 
crease of  the  tendon  reflexes,  sensitiveness  to  percussion  of 
the  entire  skull,  on  the  left  side  more  marked  than  on  the 
right,  and  choked  disc  on  both  sides. 

The  autopsy  revealed  a  soft  glioma  in  the  left  posterior 
central  convolution,  which  extended  into  the  substance  of 
the  brain. 


SYPHILITIC  CEREBRAL  MENINGITIS  93 

Until  recently  there  were  many  cases  where  it  was  im- 
possible to  make  a  differential  diagnosis  between  a  non- 
specific tumor  and  a  specific  inflammatory  process.  At  the 
present  time,  with  the  aid  of  the  four  reactions,  this  may 
be  done. 

Variation  in  the  clinical  picture  is  more  apt  to  be  found 
where  gummatous  infiltration  exists  than  where  there  is  a 
true  gummatous  tumor.  However,  one  should  remember 
that  a  non-specific  tumor  can  likewise  cause  considerable 
variation  in  its  symptoms  when  its  growth  is  slow,  so  that 
the  surrounding  nerve  tissue  has  time  to  adjust  itself  to  the 
changing  conditions,  or  through  rapidly  occurring  retro- 
grade metamorphosis,  which  also  may  take  place  in  non- 
specific growths,  or  by  hemorrhages  which  quickly  or  slowly 
change  the  character  of  the  new  growth. 

Brain  Abscess. — What  has  been  said  in  regard  to  the  dif- 
ferential diagnosis  of  brain  tumors  applies  also  to  brain 
abscess.  In  brain  abscess,  however,  another  factor  is  to  be 
considered.  One  should  never  diagnose  a  brain  abscess 
unless  an  adequate  etiological  cause  exists,  such  as  a  pri- 
mary collection  of  pus  somewhere  in  the  body,  as  occurs  in 
disease  of  the  ear  and  nose  or  after  a  head  injury. 

In  this  connection  the  following  case  is  of  interest : 

A  blacksmith,  forty-two  years  old,  had  acquired  lues 
ten  years  before,  and  sixteen  years  before  had  had  an  ear 
abscess.  He  was  taken  sick  with  general  apathy,  headache, 
a  right-sided  paresis,  and  the  gradual  development  of  a 
double  optic  neuritis.  Mixed  treatment  did  him  no  good. 
A  slight  disturbance  of  co-ordination  of  the  trunk  muscu- 
lature, together  with  a  left-sided  anosmia,  indicated  involve- 
ment of  the  frontal  lobes.  The  ears  were  normal. 

The  autopsy  revealed  a  large  encapsulated  chronic  ab- 
scess in  the  left  frontal  brain  and  an  old  osteomyelitis  of  the 
petrous  portion  of  the  temporal  bone  on  the  left  side. 

Specific  Arteritis. — Whether  or  not  the  irritation  and 
paralytic  symptoms  depend  upon  the  non-specific  effects  of 
specific  arterial  disease  is  often  difficult  to  determine,  be- 
cause thrombosis  of  the  basal  arteries,  and  the  resultant 
anaemia  thus  produced  in  the  corresponding  brain  areas, 


94 

may  cause  symptoms  of  motor  irritation.  In  many  cases 
a  marked  sensitiveness  to  percussion  of  the  skull,  and  the 
cortical  character  of  the  irritative  manifestations,  speak 
in  favor  of  a  ineningeal  affection.  One  should  not  forget, 
however,  that  frequently  both  forms  of  specific  brain-dis- 
ease, the  arteritic  and  meningitis  of  the  convexity,  exist  at 
the  same  time. 

Paresis. — In  connection  with  the  diffuse  form  of  menin- 
gitis of  the  convexity,  which  occurs  without  localizing 
symptoms,  and  not  infrequently  either  slowly  or  rapidly 
runs  the  course  of  a  progressive  dementia,  in  addition  to 
alcoholism,  the  dementia  form  of  general  paresis  must  be 
considered. 

In  chronic  alcoholism  one  often  finds  a  slight  persistent 
dementia  with  myosis  and  sluggish  reaction  of  the  pupils. 
A  clear-cut  and  permanent  loss  of  the  pupil  reflexes  would 
argue  strongly  in  favor  of  paresis. 

Both  Mendel  and  Kraft  Ebbing  have  called  our  atten- 
tion to  the  fact  that  in  the  simple  dementia  type  of  paresis 
anomalies  of  the  pupil  may  not  appear  until  late  in  the  dis- 
ease, while,  on  the  other  hand,  diffuse  cortical  brain  lues 
may  early  cause  pupillary  changes,  which  renders  a  differ- 
ential diagnosis  still  more  difficult. 

Cerebral  Arteriosclerosis. — If  the  patient  has  attained  the 
age  of  arteriosclerosis,  not  infrequently  it  is  hard  to  decide 
whether  a  diffuse  arteriosclerosis,  cortical  degeneration,  or 
a  diffuse  specific  disturbance  of  the  convexity  exists.  A  pal- 
pable arteriosclerosis  or  the  evidence  of  this  disease  in  the 
heart  does  not  prove  its  existence  in  the  cerebral  arteries, 
for  we  know  arteriosclerosis  may  appear  as  an  entirely 
localized  affection.  We  also  know  that  the  arteriosclerosis 
of  old  age,  to  be  sure  rarely,  can  cause  epileptic  attacks  of 
a  purely  Jacksonian  character,  and  even  the  development 
of  a  status  hemi-epilepticus. 

Tuberculosis. — Tuberculosis  on  the  brain  convexity  must 
also  be  considered  in  a  differential  diagnosis.  We  know 
that  this  affection  frequently  begins  with  transient  aphasia, 
cortical  irritation  and  symptoms  of  paralysis;  that  incom- 
plete paresis  can  occur,  and  that  general  symptoms  in  the 


SYPHILITIC  CEREBRAL  MENINGITIS  95 

form  of  headache,  nausea,  vomiting,  vertigo,  and  psychic 
disturbances  may  precede  the  local  symptoms,  and  that 
comparatively  long  remissions  may  also  take  place  here  as 
well  as  in  specific  disease. 

In  such  cases  the  general  habitus  of  the  patient,  also  the 
presence,  on  the  one  hand,  of  demonstrable  tuberculosis, 
and,  on  the  other,  of  present  or  past  syphilis,  the  rapid 
and  unfavorable  course  in  the  tubercular  affection  and  the 
effect  of  the  therapy,  will  enable  one  to  make  the  differential 
diagnosis. 


V 
SPECIFIC  BASILAE  MENINGITIS 

SYPHILIS  of  the  brain-base  is  more  frequent  than  of  the 
convexity.  The  base  of  the  brain  is  the  favorite  localization 
for  lues  when  it  attacks  the  nervous  system.  When  we 
also  consider  the  frequency  of  tuberculosis  on  the  brain- 
base  we  must  come  to  the  conclusion  that  this  region  of  the 
brain  offers  a  favorable  soil  for  the  development  of  the 
germs  of  disease.  Kumpf  considers  that  the  blood-vessels 
entering  into  and  going  out  of  the  brain  in  this  position, 
together  with  the  lymph-channels,  produce  for  the  growth  of 
microbes  particularly  suitable  conditions.  He  also  calls 
attention  to  the  wide  gaps  in  the  arachnoid  at  the  side  of 
sella  turcica  and  the  optic  chiasm,  as  well  as  in  the  region 
<ff  the  lamina  perforata  anterior,  as  points  of  easier  access 
to  germ  invasion. 

The  general  symptoms  are  the  same  as  occur  in  every 
chronic  and  subacute  organic  brain  disease,  and  have  been 
enumerated  in  the  description  of  the  clinical  symptoms  of 
specific  arteritis,  and  meningitis  on  the  convexity. 

Clinical  Symptoms. — Headache  is  almost  always  present, 
and  may  be  especially  severe.  It  is  most  frequently  accom- 
panied by  a  feeling  of  severe  pain,  deep  in  the  orbits  of 
the  eye.  The  sensitiveness  to  percussion  is  often  localized 
upon  the  forehead  and  over  the  eyebrows. 

Vomiting  and  vertigo  are  also  of  frequent  occurrence. 

Optic  neuritis  and  choked  discs  occur  more  often  in 
the  basal  type  than  in  any  of  the  other  forms  of  meningitis, 
and  may  be  regarded  as  evidence  of  a  lesion  of  the  optic 
nerve  in  some  part  of  its  course. 

Psychic  Disturbances. — Psychic  disturbances  manifest 
themselves  in  basilar  lues,  most  often  as  stupor,  or  as  more 
or  less  severe  conditions  of  excitement.  Well-developed 
psychoses,  whether  in  the  form  of  paranoia  or  as  melan- 
cholia or  mania,  must  be  considered  as  combinations. 

96 


SPECIFIC  BASILAR  MENINGITIS  97 

Temperature. — The  temperature,  as  a  rule,  does  not  rise 
above  the  normal.  Oppenlieim,  after  a  review  of  the  litera- 
ture of  this  subject,  came  to  the  conclusion  that  slight  tem- 
perature variations  of  atypical  character  occur  not  infre- 
quently, but  that  the  presence  of  a  high  temperature  would 
indicate  a  complication.  Quincke  reports  two  cases  with 
long-continued  subnormal  temperature. 

Polyuria  and  Polydipsia. — Polyuria  and  polydipsia,  as  in 
brain  tumor  and  after  brain  injury,  occur  in  brain  syphilis. 
Our  present  pathological  teachings  do  not  bear  out  our  pre- 
vious ideas,  that  these  symptoms  are  indicative  of  an  in- 
volvement of  the  medulla  or  its  proximity.  We  only  know 
that  polyuria  and  polydipsia  can  occur  by  such  involvement, 
but  that  both  of  these  symptoms  are  most  frequently  met 
with  in  cases  which  must  be  regarded  as  diffuse  basilar 
meningitis. 

The  Cranial  Nerves. — The  cranial  nerves  may  be  individ- 
ually attacked,  and  also  only  on  one  side.  This  is  rare, 
however.  The  rule  is  that  several  cranial  nerves  situated 
anatomically  in  close  relationship  are  involved  in  the  dis- 
eased process,  and  that  both  sides  of  the  base,  as  in  tuber- 
culosis, are  affected. 

Involvement  of  the  Olfactory  Nerve. — Cases  are  reported 
in  the  literature  of  an  affection  of  the  olfactory  nerve  where 
the  sense  of  smell  was  either  diminished  or  destroyed.  Such 
cases  would,  in  all  probability,  be  more  frequently  noted  if 
the  sense  of  smell  was  tested  oftener.  In  one  case  in  which 
the  clinical  diagnosis  was  made  of  tumor  in  the  frontal 
lobes  the  autopsy  showed  a  gummatous  growth  and  a 
chronic  gummatous  meningitis  on  the  base  of  the  right 
frontal  lobe.  Right-sided  anosmia  was  demonstrated  in  this 
case. 

Cases  of  anosmia  have  been  reported  from  simply  in- 
creased pressure  inside  the  cranium. 

The  Optic  Nerve. — The  optic  nerve  may  be  either  pri- 
marily or  secondarily  affected.  Secondary  affection  is  the 
most  frequent. 

By  primary  involvement  we  understand  that  the  nerve 
substance  itself,  without  any  extension  from  the  meninges, 

•'      7 


98 


SYPHILIS  AND  THE  NERVOUS  SYSTEM 


is  attacked  either  by  gummata  or  Heubner's  arteritis.  By 
far  the  most  frequent  points  of  involvement  in  the  optic 
tract  are  the  optic  nerve  and  the  optic  cliiasm. 

The  optic  nerve  is  more  often  secondarily  affected  than 


FIG.  30. — Transverse  section  through  a  complete  gummatous  degeneration,  and  greatly  thick- 
ened trunk  of  the  oculomotorius.    Very  few  fibres  are  preserved. 

primarily.  It  may  be  compressed  through,  gummatous  pro- 
liferations, or  suffer  by  an  infiltrating  meningitis.  It  hap- 
pens not  infrequently  that  the  gummatous  inflammation 
penetrates  in  between  the  nerve-fibres  and  causes  an  inter- 


FIG.  31. — Transverse  section  through  the  base  of  the  brain  just  in  front  of  the  optic  chiasm. 
The  intracranial  optic  trunks  are  partially  atrophied  and  entirely  enveloped  in  gummatous 
proliferations.  Syphilitic  endarteritis.  (Uhthoff.) 

stitial  syphilitic  neuritis  which  in  itself  produces  a  second- 
ary atrophy  of  the  optic  nerve-fibres. 

The  optic  nerve  can  almost  entirely  degenerate  as  in  the 
above  cut  (Fig.  30)  taken  from  Uhthoff,  or  the  nerve- 


SPECIFIC  BASILAR  MENINGITIS 


99 


fibres  themselves  may  become  infected  through  an  exten- 
sion of  the  inflammation  from  the  meninges,  which  leads 
indirectly  to  primary  parenchymatous  degeneration. 

Disease  of  the  optic  tract  from  the  bulb  to  the  primary 
optic  ganglion  manifests  itself  first  through  ophthalmo- 
scopic  changes  and  second  through  disturbances  of  the  vis- 
ion. The  ophthalmoscopic  changes  consist  in  optic  neuritis 
and  choked  disc.  We  have  seen  that  choked  disc  comes 


FIG.  32. — Transverse  section  through  the  intracranial  optic  trunk,  perineuritis  and  neuritis 

gummosa. 

occasionally  in  gummatous  meningitis  of  the  convexity.  It 
is  oftener  encountered,  however,  in  gummatous  basilar 
meningitis. 

In  a  differential  consideration  one  must  keep  in  mind 
the  frequency  of  choked  discs  in  brain  tumor,  also,  though 
less  frequent,  its  occurrence  in  apoplexy,  brain  abscess, 
albuminuria,  diabetes,  and,  in  exceptional  instances,  in 
leukaemia. 

Ophthalmoscopic  Changes  in  Choked  Disc. — In  a  true 
choked  disc  one  finds  a  precipitous  swelling  of  the  head  of 
the  optic  nerve,  the  blood-vessels  are  tortuous,  the  veins  are 
dilated,  and  the  arteries  contracted. 

Usually  hemorrhages  of  varying  degree  are  found  in  the 
retina,  in  later  stages  also  not  infrequently  whitish  or  yel- 


100  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

lowisli  areas  of  degeneration  occur  very  similar  to  those 
found  in  Bright 's  disease. 

The  condition  of  choked  disc  may  entirely  recede,  the 
papilla  again  becoming  normal,  or  it  may  end  in  the  white 
atrophy  of  the  papilla. 

Explanation  of  Choked  Disc. — The  explanation  of  Manz  in 
regard  to  the  origin  of  choked  disc  is  the  one  accepted  at  the 
present  time.  This  explanation  has  since  been  confirmed 
by  the  recent  pathological  studies  of  Saenger  and  Bier. 
This  is  that  the  fluid  from  the  subdural  and  subpial  spaces 
of  the  brain  penetrates  into  the  corresponding  spaces  of  the 
optic  nerve,  and  thus  directly  compresses  the  intra-ocular 
end  of  the  nerve. 

Optic  Neuritis. — In  syphilis  of  the  optic  nerve  conditions 
are  present  for  both  a  pure  mechanical  compression  and  an 
interstitial  and  parenchymatous  inflammation.  In  practice 
one  finds  even  in  brain  tumors  frequently  a  combination  of 
pure  choked  disc  and  inflammatory  symptoms. 

In  the  combination  of  choked  disc  and  optic  neuritis  we 
see  the  papilla  swollen,  its  contour  indistinct.  It  is  hyper- 
aemic,  and  in  color  varying  only  a  little  from  the  surround- 
ing retina. 

Neuritis  Descendens. — The  swelling  of  the  papilla  and  the 
congestion  in  the  veins  are  insignificant  in  character.  On 
the  other  hand,  a  cloudiness  and  swelling  of  the  tissue, 
which  extends  far  over  the  retina,  is  present,  showing  fre- 
quently hemorrhages  and  white  plaques,  which  are  apt  to 
be  more  numerous  in  the  region  of  the  macula  lutea,  as  is 
seen  in  retinitis  albuminuria.  The  weight  of  opinion  attrib- 
utes the  neuritis  descendens  to  an  inflammatory  process 
which  either  primarily  affects  the  nerve  substance  itself  or 
extends  from  the  surrounding  tissue  to  the  sheath  of  the 
nerve,  finally  involving  the  nerve. 

To  this  last  category  belongs  especially  meningitis  with 
secondary  perineuritis  and  neuritis. 

Combination  of  Different  Ophthalmoscopic  Changes. — On 
the  other  hand,  the  lymph  which,  through  pressure,  has  been 
forced  into  the  sheath  of  the  optic  nerve  can  act  upon  the 
axis  cylinder  as  an  exciter  of  inflammation.  We  then  have 


SPECIFIC  BASILAR  MENINGITIS  101 

the  double  symptoms  of  choked  disc  and  the  inflammation 
of  the  nerve-fibres  which  extend  out  of  the  retina. 

Disturbances  of  Vision. — We  know  from  experience  that 
a  very  high  degree  of  choked  disc  may  exist  without  any 
disturbances  of  the  vision  whatever,  and  only  when  partial 
atrophy  occurs  do  we  observe  a  greater  or  less  impairment 
of  the  field  of  vision,  both  centrally  and  on  the  periphery. 
When  an  antispecific  therapy  is  quickly  instituted  simple 
choked  disc,  as  well  as  pressure  neuritis,  comparatively 
soon  disappears,  and  one  then  saves  for  his  patient  the 
vision,  which,  when  processes  of  degeneration  have  devel- 
oped in  the  terminal  distribution  of  the  nerve-fibres,  must 
always  remain  greatly  impaired. 

Case  of  compression  neuritis  of  the  papilla  in  meningitis 
basalis  luetica  with  prompt  response  to  antispecific  therapy: 

A  man,  thirty-five  years  old,  was  received  in  the  hospital 
December  5,  1898.  He  had  acquired  lues  in  1894.  For 
about  one-half  year  his  memory  had  been  getting  weaker; 
now  and  then  he  had  attacks  of  dizziness,  which,  however, 
had  not  developed  into  unconsciousness.  For  two  weeks 
he  had  suffered  with  disturbances  of  vision  in  both  eyes. 
Objectively  a  bilateral,  well-marked  compression  neuritis 
was  found,  more  severe  on  the  right  side  than  on  the  left. 
The  patient  was  slightly  stuporous.  He  complained  of 
severe  headache.  The  pulse  was  retarded  to  50  or  60  beats 
per  minute.  His  temperature  was  subnormal.  Other  objec- 
tive symptoms  were  double  patellar  and  ankle  clonus.  The 
skull  was  sensitive  to  percussion,  especially  on  the  right 
side.  In  walking  the  patient  staggered  toward  the  left. 
The  left  facial  was  slightly  paretic. 

Four  days  after  the  beginning  of  antisyphilitic  treat- 
ment the  patient  showed  remarkable  improvement.  In 
three  weeks  he  was  in  very  good  condition.  His  gait  was 
normal,  his  sight  fairly  good,  his  mental  condition  appeared 
normal  and  the  neuritis  optica  had  almost  entirely  disap- 
peared. 

Form  of  Visual  Disturbance. — In  the  uncomplicated  cases 
of  neuritis  descendens  as  a  sequence  to  a  neuritis  or  peri- 
neuritis  localized  in  the  optic  nerve  the  disturbance  of  vision 


102  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

bears  a  distinct  character.  Both  central  and  peripheral 
vision  suffers.  Alexander  states  that  in  perineuritis  central 
vision  is  first  and  most  affected  because  the  fibres  which 
lie  in  the  periphery  of  the  optic  trunk  have  for  their  destina- 
tion the  macula  lutea,  the  point  of  sharpest  vision,  and 
naturally  they,  from  their  location,  would  suffer  first. 

On  the  other  hand,  in  primary  parenchymatous  disease 
of  the  optic  nerve  the  central  parts  are  first  and  most 
severely  affected,  and  those  fibres  are  distributed  on  the 
periphery  of  the  retina,  and  furnish  the  peripheral  vision. 

In  Marked  Disease  of  the  Optic  Nerve  Some  Vision  is 
Usually  Retained. — It  lies  in  the  nature  of  gummatous  proc- 
esses that  the  entire  cross  section  of  the  optic  nerve  is  not 
uniformly  affected,  and  as  a  result  of  this,  even  in  unfavor- 
able cases,  complete  blindness  does  not  often  occur,  but 
portions  of  the  field  of  vision  remain  preserved. 

Irregularities  in  Field  of  Vision. — As  a  matter  of  fact,  we 
find  in  practice  all  possible  forms  of  limitation  of  the  field 
of  vision,  both  of  peripheral  and  central  scotomas,  confirm- 
ing here  as  elsewhere  the  variability  and  irregularity  of 
symptoms  of  syphilis  of  the  nervous  system. 

Atrophy  of  the  Optic  Nerve  with  Double  Neuritis. — A 
woman,  sixty-three  years  old,  became  infected  with  syphilis 
at  the  age  of  twenty-four.  Ten  years  before  her  first  visit 
to  me  she  suffered  with  severe  pains  in  the  head  and  became 
blind.  At  this  time  she  received  no  treatment.  In  the 
course  of  about  two  months  some  power  of  vision  returned 
to  the  left  eye.  Four  weeks  before  she  came  to  me  she 
again  became  affected  with  severe  headache  and  dizzy 
attacks,  frequently  accompanied  by  vomiting. 

The  objective  examination  showed  in  the  eye  back- 
ground, on  both  sides,  the  picture  of  an  atrophy  consequent 
upon  a  descending  retrobulbar  neuritis,  less  advanced  on 
the  left  side.  On  the  right  side  the  patient  was  only  able  to 
distinguish  between  light  and  dark,  on  the  left  side  she  was 
able  with  difficulty  to  count  the  fingers  at  a  distance  of  four 
metres. 

Mixed  treatment  was  begun  right  away.  In  about  one 
week  the  headache  and  dizziness  had  diminished  in  inten- 


SPECIFIC  BASILAR  MENINGITIS  103 

sity.  Some  days  the  patient  was  almost  entirely  blind;  on 
others  she  seemed  to  be  able  to  see  fairly  well.  In  three 
weeks  the  headaches  and  dizziness  had  disappeared  and  the 
sight  in  the  left  eye  returned  to  about  what  it  had  been 
previous  to  this  attack.  A  year  later  she  was  still  in  the 
same  condition. 

This  case  is  interesting  for  several  reasons.  First:  It 
shows  that  without  specific  treatment  the  course  of  the  optic 
affection  was  an  unfavorable  one.  Second:  That  the  dis- 
ease in  both  eyes  did  not  run  a  uniform  course,  but  that  the 
process  in  the  left  eye  became  quiescent  before  the  vision 
was  severely  impaired.  Third:  That  the  patient,  in  her 
sixty-third  year,  thirty-nine  years  after  her  infection,  still 
manifested  symptoms  of  an  acute  syphilis. 

This  case  also  demonstrates  the  transient  character  of 
the  disturbances  of  vision,  likewise  the  varying  circulatory 
disturbances  in  the  much-diseased  arteries  of  the  optic 
nerve.  Heubner's  arteritis  would  best  explain  the  changing 
clinical  symptoms  with  reference  to  the  vision. 

Ophthalmoscopic  and  Perimetrical  Examination  Necessary. — 
Both  an  ophthalmoscopic  and  perimetrical  examination  is 
necessary  in  order  not  to  overlook  beginning  disease  of  the 
optic  nerve. 

In  those  cases  in  which  the  neuritis  is  localized  higher 
up  in  the  nerve-fibres,  and  the  descending  neuritis  has  not 
yet  reached  the  head  of  the  nerve,  one  will  not  be  able  with 
the  ophthalmoscope  to  discover  any  evidence  of  the  nerve 
involvement.  It  is  only  when  the  field  of  vision  is  taken 
that  the  beginning  disease  is  discovered.  Attention  has 
already  been  directed  to  the  fact  that  a  well-marked  choked 
disc  may  exist  without  any  disturbance  of  sight. 

The  Spinal  Form  of  Optic  Atrophy  in  Tabes. — The  spinal 
form  of  optic  atrophy  is  almost  always  an  accompanying 
symptom  of  tabes  dorsalis,  which  not  infrequently  may 
remain  for  a  long  time  as  an  isolated  symptom  of  the  dis- 
ease. Whenever  one  encounters  a  primary  optic  atrophy, 
tabes  should  always  be  thought  of,  and  in  the  majority  of 
cases  other  symptoms  will  be  discovered  which  make  secure 
the  diagnosis.  Even  in  those  cases  where  there  are  no 


104  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

evidences  of  degeneration  of  the  posterior  columns  of  any 
kind  whatever  one  may  still  feel  justified  in  the  assumption 
of  a  beginning  tabes.  The  following  case  is  of  this  nature : 

In  this  case  a  merchant,  forty-eight  years  old,  twelve 
years  after  his  infection  with  syphilis,  was  stricken  with  an 
arteritic  hemiplegia.  During  the  past  three  years  he  has 
been  afflicted  with  a  primary  optic  atrophy,  commencing 
first  on  the  right  side  and  then  involving  the  left  side,  and 
progressive  in  character. 

After  repeated  and  thorough  examinations  no  other 
symptoms  of  tabes  or  paresis  were  discoverable. 

A  second  case  is  important  because  it  seems  to  prove 
that  there  may  actually  be  a  primary  optic  atrophy  of  a 
true  degenerative  type  in  syphilis. 

A  man,  fifty-eight  years  old,  acquired  syphilis.  His 
primary  and  secondary  symptoms  were  both  energetically 
treated.  Four  years  after  his  infection  an  optic  atrophy, 
first  of  the  right  and  then  of  the  left  side,  set  in.  The 
atrophy  progressed  slowly  over  a  period  of  six  years,  and 
the  patient  became  almost  entirely  blind.  Death  occurred 
suddenly  with  symptoms  of  arteriosclerosis  of  the  heart. 
I  had  the  opportunity  of  observing  this  patient  repeatedly, 
and  never  discovered  any  other  somatic  symptoms  from 
either  the  brain  or  spinal  cord.  The  autopsy  showed 
(microscopically  examined)  gray  atrophy  of  the  optic  nerve 
of  the  true  degenerative  type. 

The  brain  and  spinal  cord  were  entirely  free  from  any 
further  evidence  of  disease,  both  macroscopically  and  micro- 
scopically. 

The  patient  had  an  arteriosclerosis  of  the  coronary 
arteries. 

The  question  whether  a  non-tabetic  and  isolated  pri- 
mary optic  atrophy,  of  the  character  of  a  spinal  atrophy, 
occurs  without  syphilis  has  not  yet  been  determined. 

As  experienced  ophthalmologists  claim,  and  as  I  have 
seen  in  two  cases  in  my  own  experience,  there  seem  to  occur 
occasionally,  spontaneously,  cases  of  isolated  optic  atrophy 
which  do  not  develop  later  into  tabes,  paresis,  or  multiple 
sclerosis. 


SPECIFIC  BASILAR  MENINGITIS  105 

I  am  able  to  report  two  cases,  one  in  a  gentleman,  fifty- 
four  years  old,  and  the  other  in  a  lady,  forty-four  years  old, 
who  for  ten  and  eleven  years  respectively  have  been  blind 
because  of  a  primary  atrophy  of  the  optic  nerve,  and  in 
whom  no  etiological  factor,  either  of  intoxication  or  infec- 
tion, could  be  demonstrated. 

The  examination  of  such  cases,  however,  as  these  at  the 
present  time  must  be  regarded  as  incomplete,  if  we  are 
unable  to  state  the  results  of  the  four  reactions  in  them. 

In  so  far  as  the  result  is  concerned  it  is,  of  course,  all 
the  same  whether  the  optic  nerve  is  compressed  by  a  tumor 
or  an  indurated  and  thickened  meningeal  mass.  The  effect 
will  be  either  that  of  congestion  or  of  atrophy.  The  diag- 
nosis of  a  specific  affection  must  be  made  from  the  history 
and  other  symptoms  in  the  case,  and  especially  from  the 
information  gained  by  the  four  reactions. 

Influence  of  Treatment. — The  treatment  of  choked  disc  by 
mercury  may  show  improvement  in  other  cases  than  those 
of  syphilitic  origin.  On  the  other  hand,  there  are  many 
cases  of  specific  disease  of  the  optic  nerve  which,  in  spite 
of  energetic  and  thorough  treatment,  terminate  in  an  un- 
favorable result, — that  is,  severe  disturbances  of  vision. 

The  following  case  is  an  illustration  of  the  benefit  some- 
times obtained  by  mercurial  treatment  in  cases  of  non- 
specific origin: 

A  coachman,  twenty  years  old,  was  thoroughly  examined 
by  me,  both  as  to  history  and  physically,  without  my  being 
able  to  find  the  slightest  evidence  of  any  past  or  present 
syphilis.  Two  weeks  before  coming  to  the  hospital  he 
began  with  headache  and  vomiting,  which  was  quickly  fol- 
lowed by  a  paralysis  in  the  left  upper  extremity  and  a 
weakness  of  the  left  face  musculature.  There  were  also 
well-developed  choked  discs,  tenderness  to  percussion  on 
the  right  side  of  the  skull,  the  intelligence  was  slightly 
dulled,  the  pulse  retarded,  and  occasionally  vomiting.  In  the 
course  of  the  next  ten  days,  after  the  patient's  admittance 
to  the  hospital,  the  paresis  deepened  into  a  paralysis,  the 
left  leg  became  first  paretic  and  then  paralytic,  and  a  com- 
plete loss  of  sensation  for  all  qualities  appeared  in  the 


106  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

entire  left  side  of  the  body.  The  behavior  of  the  skin  and 
tendon  reflexes  was  characteristic  of  an  organic  brain 
affection. 

I  felt  no  doubt  of  the  diagnosis  of  a  brain  tumor  of  a 
gliosarcomatous  nature,  which,  beginning  in  the  frontal 
bone,  was  growing  posteriorly  and  gradually  involving  the 
central  ganglia  and  the  internal  capsule.  When  the  symp- 
toms reached  the  intensity  above  described  I  ordered  inunc- 
tions and  potassium  iodid,  and  experienced  a  surprise,  as 
all  the  symptoms  gradually  began  to  disappear  after  the 
fourth  inunction.  After  four  weeks  the  only  evidences  re- 
maining of  the  former  symptoms  were  a  slight  tenderness 
of  the  left  upper  extremity,  with  slightly  increased  tendon 
and  diminished  skin  reflexes  on  the  same  side.  The  choked 
disc  had  disappeared. 

Pseudo-brain  Tumors. — I  saw  this  case  eight  years  ago,  in 
the  beginning  of  my  observations  concerning  the  so-called 
"pseudo-brain  tumors."  Since  then  I  have  had  an  oppor- 
tunity to  observe  fourteen  such  cases,  which  presented  the 
clinical  picture  of  either  a  tumor  of  the  cerebellum,  or  of 
the  motor  centres,  or  of  the  cerebellopontile  angle,  and 
either  receded  to  a  normal  condition  or  terminated  in  re- 
covery with  defect,  or  finally  caused  the  death  of  the  patient, 
without  the  autopsy  either  macroscopically  or  microscopi- 
cally revealing  any  lesion. 

Saenger,  a  number  of  years  ago,  described  a  series  of 
cases  in  which  the  symptoms  of  brain  tumor  existed,  where 
inunctions  had  produced  a  surprising  and  partial  improve- 
ment in  the  symptoms.  Later  the  autopsy  in  these  cases 
showed  no  traces  of  any  syphilitic  growth. 

More  frequent,  however,  than  the  cases  of  "pseudo- 
tumor  cerebri"  are  the  cases  beginning  with  vomiting  and 
headache,  which  develop  into  a  neuritis  of  the  optic  nerve 
of  the  choked-disc  character  and,  under  the  administration 
of  mercury,  recover  without  any  trace  of  lues  as  the  etio- 
logical  factor  whatever. 

I  have  observed  two  such  cases  during  the  past  year. 
Both  cases  were  those  of  young  girls,  in  whom,  by  the  most 
searching  examination,  not  the  least  suspicion  of  syphilis 


SPECIFIC  BASILAR  MENINGITIS  107 

could  be  obtained.  In  both  cases,  during  the  administration 
of  an  inunction  cure,  the  stasis  neuritis  disappeared,  in  one 
case  ending  in  complete  recovery,  in  the  other  a  partial 
atrophy. 

Changes  in  the  Visual  Power. — Variation  in  the  power 
of  vision  may  also  occur  in  brain  tumors  as  w.ell  as  in  gum- 
matous  meningitis.  Hirschberg  cites  cases  of  brain  tumor 
in  which  the  field  of  vision  half-hourly  and  hourly  narrowed 
and  widened.  A  transient  blindness  appearing  in  attacks 
and  quickly  disappearing,  which  has  been  called  epileptic 
amaurosis  (Jackson),  is,  according  to  Hirschberg,  a  fairly 
regular  accompanying  symptom  of  brain-tumor,  for  which 
one  must  search  carefully  because  of  its  fleeting  character. 

While  we  find  the  cause  of  the  variation  in  the  power 
of  vision  in  a  recurring  anaemia  of  the  sight  centres  due  to 
a  transient  increase  in  the  brain  pressure,  we  see  the  ex- 
planation of  the  more  frequent  and  more  intensive  appear- 
ing alterations  in  vision  in  specific  disease  of  the  optic 
nerve,  either  in  a  change  of  pressure  on  the  part  of  the 
compressing  growth,  or  in  the  more  quickly  terminating 
biological  conditions  of  syphilitic  granulation  tissue,  or, 
finally,  in  the  varying  behavior  of  the  lumen  of  the  affected 
arteries  of  the  nerve  itself. 

That  the  optic  nerve  is  very  sensitive  to  anaemia  is  well 
known,  and  especially  has  our  experience  with  amaurosis 
in  severe  hemorrhages  taught  us  this.  This  sensitiveness 
of  the  fibres  of  the  optic  nerve  and  their  end  processes  is 
the  more  serious  because  of  the  fact  that  each  branch  of 
the  retinal  artery  is  an  end  artery. 

Oscillation  of  Sight  in  a  Sarcoma  of  the  Pia  Sheath  of  the 
Optic  Nerve. — In  a  case  of  extensive  sarcomatous  growth  of 
the  pia  mater  of  the  entire  central  nervous  system  the 
patient,  a  seventeen-year-old  girl,  manifested  an  intense 
variation  in  her  power  of  vision.  Finally  the  sight  was  en- 
tirely destroyed.  One  could  demonstrate,  ophthalmoscopi- 
cally,  an  extreme  anaemia  of  both  papillae.  A  later  micro- 
scopic examination  of  the  optic  nerve  showed  that  the  tumor 
masses  which  had  proliferated  into  the  pia  had  greatly 


108  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

compressed,  in  places  causing  a  complete  shutting  off  of 
the  lumen  in  the  blood-vessels  in  the  optic  trunk. 

Uhthoff's  Experience  Concerning  Disease  of  the  Optic  Nerve 
in  Brain  Syphilis. — Naturally  the  experience  of  the  neurolo- 
gist in  syphilis  of  the  optic  nerve  and  eye  musculature 
would  tend  to  be  much  less  than  that  of  the  ophthalmolo- 
gist. The  co-operative  work  of  Uhthoff  on  the  one  hand, 
and  of  Oppenheim  and  Siemerling  on  the  other,  has  accom- 
plished much  in  the  field  of  syphilitic  optic  disturbances. 

Uhthoff,  from  cases  of  brain  lues  in  the  Charite  in  Ber- 
lin, found  that  only  15  per  cent,  of  the  cases  were  entirely 
free  from  eye  affections.  In  the  cases  observed  by  him  at 
the  autopsy  the  great  variation  in  the  pathological  changes 
is  well  illustrated. 

In  the  first  case,  perineuritis,  cellular  infiltration  of  the 
optic  nerves,  proliferation  and  new  growth  from  the  blood- 
vessels in  the  optic  sheath,  cell  proliferation  into  the  walls 
of  the  arteries  themselves,  and  penetrating  even  into  the 
interior  of  the  optic  nerve,  were  found. 

In  a  second  case  there  was  a  primary  gummatous  neu- 
ritis of  the  optic  trunks  and  specific  disease  of  the  vessels  in 
the  nerves. 

In  a  third  case  there  were  neuritis  and  perineuritis  in- 
terstitialis  and  the  partial  transformation  of  the  optic  tract 
into  a  gummatous  mass. 

In  the  fourth  case  disease  of  the  optic  nerve  was  an 
accompanying  symptom  in  a  gummatous  encephalomenin- 
gitis  of  both  frontal  lobes. 

In  typical  cases  reported  by  Siemerling  and  Oppenheim 
there  were  also  a  gummatous  neuritis  and  perineuritis  of 
the  other  nerves  on  the  base  of  the  brain,  gummatous  infil- 
tration or  gummatous  tumor  in  the  neighborhood  of  the 
basal  nerve  tracts  and  in  the  brain  ganglia ;  gummata  were 
also  found  in  other  portions  of  the  brain,  as  the  temporal 
lobes.  At  the  same  time,  in  many  cases  more  or  less  exten- 
sive specific  lesions  of  the  blood-vessels  and  processes  of 
softening  in  the  region  of  the  basic  ganglia  and  the  internal 
capsule  were  observed. 

It  has  also  been  discovered  that  tabes  as  a  non-specific 


SPECIFIC  BASILAR  MENINGITIS  109 

process  may  be  combined  with  a  genuine  specific  affection 
of  the  optic  nerve  (Nonne),  and  that  a  syphilis  localized 
in  the  spinal  cord  can  be  associated  with  luetic  affections 
of  the  eye,  and  particularly  of  the  optic  nerves. 

Uhthoff,  in  seventeen  autopsies,  found  an  involvement 
of  the  optic  nerve  in  fourteen  cases.  In  100  clinical  cases 
of  his  own,  in  twenty  the  optic  nerve  was  the  only  involve- 
ment. He  classifies  the  clinical  symptoms  of  the  optic 
apparatus  into  two  groups :  first,  the  ophthalmoscopic  find- 
ings, and,  second,  the  various  visual  disturbances. 

Among  100  clinical  cases  of  brain  syphilis  observed  by 
him,  he  found  double  typical  choked  disc  four  times,  neuritis 
optica  eight  times,  optic  atrophy  once.  From  150  cases  col- 
lected from  the  literature  of  cerebral  syphilis,  he  found 
choked  disc  fifteen  times,  neuritis  optica  seven  times,  and 
simple  atrophy  ten  times.  It  should  also  be  pointed  out 
here  that  Uhthoff  in  five  cases  of  disease  of  the  optic  nerve, 
centrally  localized,  was  not  able  to  demonstrate  any  evi- 
dence of  an  affection  of  the  nerve  ophthalmoscopically. 

These  cases  illustrate  what  has  already  been  pointed  out, 
namely,  the  necessity  in  any  case  of  a  testing  of  the  vision 
of  the  eye  in  addition  to  the  ophthalmoscopic  examination. 

In  Uhthoff 's  material  the  pathological  basis  for  the 
choked  disc  was  found  in  hydrops  of  the  sheath  of  the  optic 
nerve,  with  perineuritic  changes  in  the  space  between  the 
sheath  and  the  nerve,  in  encephalomeningitis,  and  also  in 
gummatous  proliferations  in  this  space. 

Choked  Disc. — Only  in  one  case  of  Uhthoff 's  was  the 
choked  disc  limited  to  one  side.  In  all  others  it  was  bilat- 
eral. It  was  also  his  experience  that  choked  disc  in  brain 
syphilis  can  entirely  disappear  without  leaving  behind  any 
disturbances  of  function  worth  mentioning. 

In  one  case  in  which  a  high  degree  of  choked  disc  had 
appeared  for  the  second  time,  one  side  cleared  up  com- 
pletely, while  the  other  went  on  to  simple  atrophy  and  total 
loss  of  vision.  Now  and  then  choked  disc  may  remain 
for  a  long  time  the  only  symptom  of  brain  lues.  It  also 
happens  that  brain  syphilis,  after  the  neuritis  optica  has 


110  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

caused  a  more  or  less  severe  disturbance  of  the  function  of 
sight,  becomes  quiescent. 

Optic  Neuritis. — Uhthoff  found  from  his  own  material 
neuritis  optica  without  choked  disc  in  12  per  cent,  of  the 
cases,  from  150  cases  in  the  literature  in  6  per  cent.  The 
neuritis  was  caused  in  these  cases  by  perineuritic  changes 
of  the  orbital  optic  trunks,  basilar  gummatous  meningitis, 
and  specific  tumor  formation.  Hutchinson  reports  specific 
disease  of  the  optic  nerves  in  one  case. 

Optic  Atrophy. — Simple  optic  atrophy  occurred  in 
Uhthoff 's  100  cases  fourteen  times,  in  four  of  which  the 
atrophy  was  complete,  in  the  others  only  partial.  In  the 
150  cases  taken  from  the  literature,  atrophy  occurred  ten 
times. 

Visual  Disturbances. — In  regard  to  the  disturbances  of 
vision,  from  Uhthoff 's  statistics  and  a  review  of  the  litera- 
ture one  learns  that  they  may  develop  either  quickly  or 
slowly,  that  they  are  usually  incomplete,  and  that  they  show 
a  variation  in  their  course,  often  are  one-sided,  and  that  the 
field  of  vision  is  of  different  types.  Uhthoff  saw,  in  his 
large  experience,  a  double-sided  complete  and  permanent 
blindness  only  once,  twice  a  transient  complete  blindness  on 
both  sides,  and  seven  times  a  complete  and  permanent  blind- 
ness of  one  eye. 

Brain  lues,  in  contradistinction  to  tumor  cerebri  and  the 
different  forms  of  non-specific  cerebral  meningitis  and  hy- 
drocephalus,  causes  total  loss  of  vision  only  in  rare 
instances. 

Chiasm  and  Optic  Tract. — The  optic  tract  and  the  optic 
chiasm  are  affected  by  luetic  processes  still  more  often  than 
the  optic  nerves.  The  optic  chiasm  is  usually  primarily 
affected,  and  from  there  as  a  starting  point  the  specific 
process  advances  either  backward  along  the  optic  tract  or 
forward  to  the  optic  nerves.  Disease  of  the  chiasm  in  addi- 
tion to  the  ophthalmoscopic  changes  may  be  recognized  by 
the  hemianoptic  visual  disturbances.  From  the  assumption 
of  a  partial  crossing  of  the  optic  fibres  in  the  chiasm  as  a 
fact,  bitemporal  and  binasal  hemianopsia  are  explained 
when  the  anterior  or  posterior  angle  of  the  chiasm  is 


SPECIFIC  BASILAR  MENINGITIS 


111 


affected,  and  homonymous  hemianopsia.  occurs  if  the  optic 
tracts  are  involved.  One  can  readily  understand  that  prac- 
tically all  sorts  of  complications  and  transitions  occur.  As 
an  example,  if  one  branch  of  the  optic  tract  is  diseased,  and 
from  this  point  there  is  an  extension  of  the  affection  to  the 
chiasm,  in  addition  to  a  liomonymous  hemianopsia  a  pro- 
gressive disturbance  of  vision  must  develop  for  the  other 
half  of  the  field  of  vision. 

Only  very  rarely  are  hemianoptic  disturbances  caused 
by  a  shutting  off  of  the  blood-supply  to  the  basal  part  of  the 
optic  conduction  apparatus. 


FIG.  33. — Cross  section  of  the  chiasm  not  far  from  the  anterior  angle,  gummatous  prolifera- 
tions, endarteritis.     (Oppenheim.) 

One  may  conclude,  if  the  visual  defect  starts  as  hemian- 
opsia, the  pathological  process  has  begun  either  in  the 
chiasm  or  in  the  tracts;  on  the  other  hand,  if  the  visual 
defect  begins  as  purely  one-sided,  the  process  has  had  its 
beginning  in  the  optic  nerve  on  one  side  or  the  other,  and 
from  this  point  extended  to  the  chiasm.  Uhthoff  estimates 
that  10  per  cent,  of  all  cases  of  homonymous  hemianopsia 
and  15  per  cent,  of  all  cases  of  bitemporal  hemianopsia.  are 
of  specific  origin. 

Homonymous  hemianopsia  is  more  frequent  than  hete- 
ronymous. 

Uhthoff    from    his    material    found    the    homonymous 


112  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

hemianopsia  eleven  times  and  the  bitemporal  form  of  the 
heteronymous  type  six  times.  In  all  of  his  cases  he  did 
not  observe  a  single  case  of  the  binasal  type,  and  from  the 
150  cases  in  the  literature  only  one  case  was  reported. 
Concentric  narrowing  of  the  visual  field  he  observed  five 
times. 

Oscillating  Hemianopsia. — Oppenheim  has  described  an 
oscillating  form  of  hemianopsia  or  hemianopsia  fugax  as 
characteristic  in  basilar  brain  lues.  He  has  observed  three 
such  cases.  In  a  period  varying  from  two  days  to  two 
weeks  the  area  of  the  bitemporal  visual  field  changed 
quickly,  enlarging  and  diminishing. 

In  the  following  case  the  type  of  the  visual  field  per- 
mitted the  localization  of  the  process  to  the  chiasm;  also 
a  right-sided  facial  paralysis  indicated  a  lesion  on  the  base. 

Basal  Bitemporal  Hemianopsia,  Descending  Neuritic  Atrophy 
on  One  Side. — A  man,  forty-six  years  old,  who  twelve  years 
before  had  acquired  syphilis,  was  taken  without  any  appar- 
ent cause  with  a  feeling  of  pressure  and  heaviness  over 
both  eyes.  He  did  not  have  any  headache,  vomiting,  or 
dizziness.  Two  weeks  later  he  observed  a  shadow  on  the 
right  side  of  the  right  eye.  The  sight  of  the  right  eye  grad- 
ually diminished.  Examination  demonstrated  that  in  the 
right  eye  the  patient  was  hardly  able  to  distinguish  the 
light,  the  right  pupil  was  smaller  than  the  left  and  did  not 
react  to  the  light,  while  the  consensual  and  convergence 
reactions  were  present.  The  right  papilla  was  only  a  little 
paler  than  the  left.  The  left  pupil  reacted  normally  and 
the  vision  in  it  was  normal.  There  was  no  paralysis  of  the 
eye  muscles  on  either  side. 

Examination  of  the  field  of  vision  revealed  an  almost 
total  amaurosis  of  the  right  eye ;  in  the  left  eye  almost  half 
of  the  field  of  vision  on  the  temporal  side  was  affected. 

As  the  result  of  antispecific  treatment,  after  five  months 
an  examination  of  the  field  of  vision  demonstrated  in  the 
left  eye  still  a  slight  defect  in  the  external  upper  quadrant ; 
in  the  right  eye  the  visual  disturbance  involved  the  entire 
temporal  half  of  the  field  of  vision  and  the  upper  half  of 
the  field  of  vision  on  the  nasal  side. 


SPECIFIC  BASILAR  MENINGITIS  113 

Eleven  months  later  I  saw  this  patient  again.  Four 
months  before  he  had  developed  a  maniacal  condition,  which 
afterward  changed  to  severe  depression.  Objectively  the 
patient  was  much  depressed.  Both  pupils  were  somewhat 
dilated  and  the  reaction  to  light  was  a  little  weaker  on  the 
right  side  than  on  the  left.  With  the  consensual  reflex  the 
reverse  was  true.  The  right  eye  was  amaurotic  and  the 
ophthalmoscope  showed  a  total  optic  atrophy.  The  right 
facial  was  paralyzed  in  all  its  branches.  The  skull  was 
moderately  sensitive  to  percussion. 

The  patient  did  not  react  this  time  to  treatment,  the 
depression  remained,  and  two  months  later  he  died  of 
pneumonia. 

Basal  Homonymous  Left-sided  Hemianopsia,  Together  with 
Abducens  Paralysis  on  the  Right  Side. — This  patient,  a,  woman, 
twenty-seven  years  old,  I  saw  first  on  December  17,  1893. 
She  had  been  infected  with  syphilis  about  eight  months 
before,  and  had  taken  a  course  of  inunctions.  Two  weeks 
before  I  saw  her  she  was  seized  with  severe  headache  which 
made  her  stupid,  and  was  most  intense  over  the  eyes.  For 
two  days  she  had  double  vision  and  had  a  feeling  as  of  a 
mist  or  veil  before  her  eyes. 

A  rather  marked  tenderness  to  percussion  was  present 
over  the  entire  cranium,  most  marked,  however,  over  the 
forehead  and  above  the  eyes.  The  right  abducens  was 
paretic;  the  remaining  eye  muscles,  including  the  pupil- 
lary, functionated  normally. 

In  the  background  of  the  eye  one  could  demonstrate  a 
moderate  degree  of  congestion-neuritis  on  both  sides. 

The  perimetric  examination  revealed  a  large  left-sided 
hemianoptic  defect.  After  six  weeks  of  treatment  the 
patient  left  the  hospital.  A  small  hemianoptic  defect  of  the 
field  of  vision  still  existed.  The  optic  neuritis  had  disap- 
peared. 

Hemioptic  Reaction  of  the  Pupils. — A  symptom  for  the 
localization  of  an  affection  in  one  or  the  other  of  the  optic 
tracts  has  been  recognized  since  Wernicke,  in  1883,  directed 
our  attention  to  the  hemioptic  reaction  of  the  pupils.  The 
symptom  of  Wernicke  may  be  explained  in  this  way :  Hemi- 

8 


114  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

optic  reactions  are  obtained  if  the  reaction  to  light  of  both 
pupils  remains  unimpaired  when  both  halves  of  the  retina, 
which  are  light-sensitive,  are  illuminated ;  on  the  other  hand, 
the  reaction  is  lost  when  the  light  strikes  the  halves  of  the 
retina  from  which  the  vision  is  lost.  Ley  den  was  the  first, 
in  1892,  to  confirm  Wernicke's  teaching  by  the  report  of  a 
pertinent  case  with  autopsy.  Oppenheim  acknowledges  in 
his  book  on  neurology  the  truth  of  Wernicke's  theory,  but 
emphasizes  the  fact  that  in  practice  proof  of  the  hemioptic 
loss  of  the  reaction  of  the  pupils  to  light  in  convincing 
clearness  is  rarely  obtained. 

The  following  is  the  report  of  a  case  of  hemioptic  loss 
of  the  light  reflexes  which,  together  with  other  symptoms, 
enabled  me  to  correctly  diagnose  a  specific  affection  of  the 
optic  tract. 

Hemianopsia,  Hemianoptic  Pupil  Reaction,  Basal  Paralysis 
of  the  Eye  Musculature,  and  Epilepsy. — A  laborer,  forty-three 
years  old,  was  under  my  observation  in  the  hospital  four 
different  times.  Six  years  before  his  first  entrance  into 
the  hospital,  which  was  January  21, 1896,  he  had  contracted 
syphilis.  About  a  year  before  he  came  into  the  hospital  he 
became  affected  with  a  severe  headache  and  noticed  that  his 
power  of  vision  suffered  in  this  manner.  It  was  difficult 
for  him  to  recognize  objects  on  his  left. 

At  the  first  examination  a  paresis  of  the  left  rectus 
internus,  a  left  facial  paresis  peripheral  in  character,  and 
diminished  capacity  for  smell  on  the  left  side  were  found. 
The  pulse  was  60.  Ophthalmoscopically  there  was  nothing 
definite  to  determine;  on  the  other  hand,  the  examination 
of  the  visual  fields  demonstrated  a  left-sided  hemianoptic 
defect. 

Under  treatment  all  the  symptoms  disappeared  with  the 
exception  of  the  visual  defect.  Six  months  later  the  patient 
was  again  received  into  the  hospital  because  he  had  such  a 
severe  headache,  the  visual  disturbance  on  the  left  side 
had  increased,  and  he  also  had  convulsive  attacks.  The 
convulsions  were  epileptic  in  nature,  occurred  both  by  day 
and  night,  and  were  accompanied  with  biting  of  the  tongue, 
the  passing  of  urine,  and  left  behind  them  amnesia. 


SPECIFIC  BASILAR  MENINGITIS  115 

Objectively  there  was  a  total  kemianopsia  sinistra;  the 
temporal  half  of  the  left  papilla  was  paler  than  the  nasal 
half.  When  one  carefully  (in  a  dark  room)  allowed  the 
light  to  fall  on  the  temporal  half  of  the  right  and  nasal  half 
of  the  left  papilla,  no  light  reaction  could  be  obtained,  while 
the  reaction  promptly  occurred  when  the  other  retinal 
halves  were  stimulated  with  the  light. 

Antisyphilitic  treatment  of  three  months'  duration  did 
not  show  any  results. 

At  a  third  entrance  into  the  hospital  the  objective  ex- 
amination was  the  same. 

A  year  later  the  patient  was  again  taken  into  the  hos- 
pital because  of  a  left-sided  hemiparesis  which  suddenly 
developed  like  an  apoplexy.  The  paresis,  under  antispecific 
treatment,  almost  entirely  disappeared.  The  ophthalmo- 
scopic  examination  was  the  same  as  before.  The  hemian- 
optic  loss  of  the  pupillary  reflexes  to  light  could  also  be 
demonstrated. 


VI 

SYMPTOMATOLOGY  OF  SYPHILIS  OF  THE  BASE 
OF  THE  BRAIN 

The  Frequency  of  Paralysis  of  the  Eye  Muscles  in  Lues  of 
the  Nervous  System. — Paralysis  of  the  nerves  which  supply 
the  eye  muscles  is  especially  frequent  in  syphilis  of  the 
nervous  system.  Grafe  says  that  more  than  half  of  all  the 
paralyses  of  the  eye  muscles  are  of  specific  origin.  Alex- 
ander, in  his  monograph  "Syphilis  and  the  Eye,"  states 
that  from  269  cases  of  eye-muscle  paralysis  observed  by 
him,  53.5  per  cent,  were  of  syphilitic  nature.  Callus  and 
Wilde  found  in  141  cases  of  eye-muscle  paralysis  syphilis 
to  be  the  cause  of  one-third  of  them. 

A  glance  at  the  anatomical  relations  in  order  to  under- 
stand the  frequency  of  paralysis  of  the  eye  musculature  will 
not  be  out  of  place  here. 

Course  of  the  Oculomotor  Nerve. — The  oculomotor,  accord- 
ing to  Gegenbaur,  leaves  the  brain  just  in  front  of  the  pons 
and  towards  the  centre  from  the  crura,  runs  between  the 
arteria  -cerebelli  superior  and  inferior  anteriorly,  and  lat- 
erally to  the  side  of  the  posterior  clinoid  process,  where  it 
passes  through  the  dura  and  enters  into  the  upper  wall  of 
the  sinus  cavernosus.  Here  it  lies  on  the  last  bend  of  the 
internal  carotid  and  reaches  the  orbits  through  the  superior 
orbital  fissure.  Before  its  entrance  into  the  orbital  fissure 
it  divides  into  two  branches,  both  of  which  lie  just  at  the 
side  of  the  optic  nerve.  The  region  in  which  the  oculomotor 
nerve  courses  is  one  whose  structures,  the  pons,  crura, 
blood-vessels,  and  bony  surfaces  of  the  orbital  fissure,  are 
particularly  inclined  to  syphilitic  processes. 

The  nucleus  of  the  nerve  is  also  unusually  extended,  for 
it  reaches  from  the  posterior  end  of  the  third  ventricle 
underneath  the  anterior  corpora  quadrigemina  up  to  and 
beneath  the  posterior  corpora  quadrigemina.  It  consists  of 
a  large  number  of  large  and  small  ganglion  cells,  which  are 

116 


SYPHILIS  OF  THE  BASE  OF  THE  BRAIN  117 

arranged  group-like  in  relation  to  one  another  and  permit 
of  a  division  of  the  entire  nucleus  into  several  subdivisions. 
Subdivisions  of  the  Oculomotor  Nucleus. — In  regard  to  the 
location  of  the  various  subdivisions  of  the  oculomotor 
nucleus,  it  seems  to  be  definitely  settled  that  the  nuclei  sup- 
plying the  sphincter  pupillae  and  the  ciliary  muscle  lie  the 
farthest  forward,  that  the  nucleus  supplying  the  levator 


FIG.  34. — Scheme  of  the  oculomotor  (1-8)  and  trochlear  (9)  nuclei.  (According  to  Perlia.) 
1-2  (anterior  smaller),  3-8  (posterior  larger),  subdivisions  of  the  oculomotor  nucleus;  8,  central 
nucleus;  4-7,  lateral  subdivisions;  4  and  5  (dorsal),  6  and  7  (central),  parts  of  the  lateral  sub- 
division; 3,  nucleus  of  Edinger  and  Westphal;  1,  fibres  for  the  pupillary  movements  through 
the  sphincter  pupillae;  2,  fibres  for  the  pupillary  movements  through  the  levator  palpebre 
superioris;  3,  fibres  for  the  pupillary  movements  through  the  convergence  and  accommodation; 
4,  fibres  for  the  pupillary  movements  through  the  rectus  superior;  5,  fibres  for  the  pupillary 
movements  through  the  obliquus  inferior;  6,  fibres  for  the  pupillary  movements  through  the 
rectus  inferior;  7,  fibres  for  the  pupillary  movements  through  the  rectus  interims.  9  is  the 
schematic  representation  of  the  trochlear  nucleus, 

palpabrae  superioris  lies  anteriorly  to  the  nuclei  of  the 
other  voluntary  eye  muscles,  and  that  the  Westphal  group 
is  connected  with  the  accommodation. 

These  anatomical  conditions  explain  a  priori  the  occur- 
rence of  isolated  paralyses  of  the  individual  internal  and 
external  eye  muscles.  t 


118 


SYPHILIS  AND  THE  NERVOUS  SYSTEM 


The  Blood  Supply  of  the  Oculomotor. — The  trochlear,  ab- 
ducens,  and  oculomotor  nerves  not  only  receive  their  blood 
supply  from  different  sources,  but  the  oculomotor  alone  is 
nourished  from  two  separate  and  distinct  regions,  the  an- 
terior portion  being  supplied  by  the  arteria  cerebri  pos- 
terior, and  the  posterior  from  branches  given  off  directly 
from  the  basilar  artery.  For  this  reason  the  nucleus  of  the 
oculomotor  nerve  is  frequently  affected  with  isolated  areas 
of  disease  which  are  produced  by  the  involvement  of  a 
single  artery. 

Fig.  35  represents  the  schematic  plan  of  the  blood  supply 
of  the  oculomotor. 

Shiamura  calls  attention  to  another  reason  why  a  vascu- 
lar explanation  for  the  frequency  of  disease  of  this  nucleus 
seems  probable.  The  nucleus  is  situated  in  the  brain  at  the 


FIG.  35. — Plan  of  the  blood  supply  of  the  brain-stem,  oculomotor  nucleus,  and  root  areas. 
(According  to  Rossolimo-Shiamura.)  d,  dorsal;  I,  lateral;  m,  mesial  arterial  regions;  1,  fibres 
from  the  frontal  lobes  and  the  pons;  2,  motor  tract  of  the  cranial  nerves;  3,  motor  tract  of  the 
extremities;  4,  fibres  from  the  occipital  and  temporal  lobes. 

point  where  the  streams  of  two  great  arterial  trunks,  the 
carotid  on  the  one  hand  and  the  vertebral  on  the  other,  come 
into  contact  with  each  other  through  the  agency  of  the 
arteria  communicans  posterior.  Furthermore,  all  the 
arteries  supplying  the  oculomotor  nucleus  are  end-arteries 
in  the  sense  of  Cohnheim,  and  ascend  almost  perpendicu- 
larly into  the  substance  of  the  nucleus  from  the  ventral  to 
the  dorsal  side. 


SYPHILIS  OF  THE  BASE  OF  THE  BRAIN  119 

Separate  Course  of  the  Root-fibres. — The  separation  of 
the  root  fibres  of  the  different  groups  of  the  oculomotor 
nerve  before  they  make  their  exit  out  of  the  brain  also 
furnishes  an  anatomical  basis  for  the  possibility  of  isolated 
disturbances  of  function  of  the  individual  eye  muscles. 

It  might  be  well  here  to  call  attention  to  what  both  clini- 
cal and  pathological  experience  has  proven  to  be  true; 
namely,  that  a  functional  disturbance  of  isolated  external 
(bulbomotor)  and  internal  (pupillary)  fibres  can  present 
the  clinical  picture  of  disease  of  the  oculomotor  nerve  trunk. 

Course  of  the  Trochlear  Nerve. — The  trochlear  nerve  leaves 
the  brain  posterior  to  the  corpora  quadrigemina,  winds 
around  the  crura  to  the  brain  base,  where  it  penetrates  the 
dura  on  the  medial  edge  and  anterior  portion  of  the  ten- 
torium.  With  reference  to  the  oculomotor  nerve  it  lies  lat- 
erally and  posteriorly,  and  with  reference  to  the  fifth  nerve, 
above  it.  It  runs  inside  the  dura,  parallel  with  the  sinus 
cavernosus,  and,  crossing  obliquely  the  oculomotor,  attains 
the  supraorbital  fissure,  as  the  extreme  posterior  part  of 
the  oculomotor  nucleus. 

Course  of  the  Abducens. — The  abducens  leaves  the  brain 
at  the  posterior  edge  of  the  pons,  runs  beneath  and  some- 
what medially  from  the  point  of  exit  of  the  fifth  nerve 
through  the  dura,  in  order  to  ascend  at  the  side  of  the 
dorsum  sellae.  It  enters  the  sinus  cavernosus  laterally  to 
the  internal  carotid  and  passes  through  the  superior  orbital 
fissure  into  the  orbit.  The  nucleus  lies  about  in  the  middle 
of  the  floor  of  the  fourth  ventricle. 

Is  Paralysis  of  the  Eye  Muscles  Pathognomonic  of  Lues? — 
If  one  should  ask  whether  paralysis  of  the  eye  muscles  is 
somewhat  pathognomonic  of  syphilis  of  the  nervous  sys- 
tem, I  should  feel  compelled  to  answer  in  the  negative. 
Proofs  of  syphilis  in  the  anamnesis  or  other  sure  evidence 
of  lues  must  be  found  before  a  definite  diagnosis  of  specific 
disease  can  be  made,  although  isolated  paralysis  of  one  or 
more  of  the  eye  muscles  often  occurs. 

If  the  specific  processes  are  situated  in  the  anterior 
cranial  fossa,  the  olfactory  nerve,  the  optic  nerve,  and  the 
first  branch  of  the  trigeminus  may  be  affected  and  the 


120  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

nerves  of  the  eye  muscles  will  remain  unaffected.  If  the 
disease  is  located  in  the  middle  cranial  fossa,  around  the 
hypophysis,  in  addition  to  the  nerves  of  the  eye  muscles 
the  optic  chiasm  will  also  be  involved.  In  disease  of  the 
posterior  cranial  fossa  the  eye  muscles  will  again  escape, 
and  the  cranial  nerves  most  apt  to  be  diseased  will  be  the 
trigeminus  and  the  hypoglossal  and  the  nerves  lying  be- 
tween these  two. 

The  histories,  however,  of  many  of  my  cases  show  that 
in  practice  the  above  scheme  does  not  always  hold  good,  but 
that  the  clinical  picture  of  basilar  specific  meningitis  may 
present  much  that  is  irregular. 

In  the  following  case,  a  basilar  meningitis,  which  had 
caused  an  isolated  oculomotor  paralysis,  recovered  under 
treatment,  but  died  later  of  uraemia.  The  autopsy  and 
pathological  examination,  both  macroscopical  and  micro- 
scopical, confirmed  the  diagnosis  of  a  lesion  of  the  oculo- 
motor. 

A  coachman,  thirty-three  years  old,  became  infected 
with  syphilis  in  June,  1890.  Two  months  later  in  addition 
to  well-marked  secondary  syphilis  he  developed  a  left-sided 
paralysis  of  the  third  nerve,  which  seemed  peripheral  in 
character,  because  external  and  internal  fibres  appeared 
equally  affected. 

In  1893  this  patient  took  a  course  of  inunctions  because 
of  ulcerations  on  the  lower  limbs.  In  1898  another  similar 
treatment  for  the  relief  of  cephalalgia  nocturna  and  an 
ulcer  on  the  right  forearm.  In  1899  he  was  again  admitted 
into  the  hospital  because  of  severe  headache,  diarrhoea, 
vomiting,  and  swelling  of  the  feet  and  limbs. 

Objectively,  in  so  far  as  the  nervous  system  was  con- 
cerned, particularly  the  eye  muscles,  nothing  was  to  be 
demonstrated.  The  patient,  however,  was  suffering  with  a 
severe  parenchymatous  nephritis  with  an  albumin  retinitis 
from  which  he  died  in  two  weeks. 

The  autopsy  showed  a  parenchymatous  nephritis,  and  in 
the  liver  interstitial  areas  and  multiple  gummata.  In  the 
brain  there  were  slight  changes  in  the  basal  arteries  (the 
aorta  was  intact),  there  were  no  foci  of  disease,  and  the  pia 


SYPHILIS  OF  THE  BASE  OF  THE  BRAIN  121 

mater  over  both  hemispheres  was  diffusely  clouded  and 
thickened.  On  the  base  the  pia  over  the  pons  and  region 
of  the  oculomotor  bore  a  similar  appearance,  the  other  por- 
tions of  the  base  remaining  uninvolved. 

The  microscopical  examination  of  both  nuclei  of  the 
oculomotor  demonstrated  normal  conditions,  with  no  par- 
ticular difference  between  the  right  and  left  side. 

The  following  case  was  one  of  extensive  basilar  disease 
which  involved  the  optic,  oculomotor,  and  fifth,  as  well  as 
the  abducens  and  facial  on  the  left  side. 


FIG.  36. — Cerebral  basilar  lues.     Rapid  improvement  under  mixed  treatment. 

The  patient  was  a  woman,  thirty-three  years  old,  with  a 
negative  history  in  regard  to  syphilis. 

Four  months  before,  she  was  seized  with  severe  head- 
ache, which  shortly  disappeared  without  treatment.  It  soon 
returned,  however,  and  more  intense  than  before.  The  pain 
came  in  attacks,  usually  at  night,  which  were  so  severe  that 
she  could  not  sleep.  Two  months  later  the  power  of  vision 
in  the  right  eye  became  poor.  This  visual  loss  manifested 
a  considerable  degree  of  variation  from  day  to  day.  Two 
weeks  before  her  entrance  into  the  hospital  she  saw  double. 
When  she  came  into  the  hospital,  examination  showed  that 
she  was  poorly  nourished  and  that  the  internal  organs 
were  normal. 


122  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

The  skull  was  sensitive  to  percussion  over  the  frontal 
and  parietal  regions  on  the  left  side,  likewise  the  points  of 
exit  of  the  left  supra-  and  infraorbital  nerves.  In  the  entire 
area  supplied  by  the  first  branch  of  the  fifth  a  slight  anaes- 
thesia of  the  skin  and  mucous  membrane  could  be  demon- 
strated. The  left  abducens  was  paretic,  also  the  left  facial. 
Both  pupils,  except  that  they  reacted  to  light  slowly,  ap- 
peared normal.  In  the  background  of  the  right  eye  one 
could  see  a  partial  atrophy,  in  the  left  a  slight  degree  of 
choked  disc. 

Under  hydro-  and  electrotherapy  the  symptoms  grew 
worse,  rather  than  better. 

After  a  week,  a  course  of  inunctions  and  potassium 
iodid  internally  was  begun.  The  result  was  surprising. 
The  headache  had  improved  by  the  third  day,  and  by  the 
fifth  day  the  abducens  and  facial  paresis  had  disappeared. 
Four  weeks  later  anaesthesia  remained  only  in  the  first 
branch  of  the  fifth.  The  second  and  third  branches  were 
normal  in  their  function.  In  the  right  eye  the  optic  atrophy 
remained,  but  in  the  left  the  papilla  appeared  normal. 

The  following  case  is  an  example  of  a  left-sided  facial 
and  auditory  nerve  disease  accompanied  by  a  right-sided 
hemiplegia : 

Meningitis  Basalis  Luetica. — A  shoemaker,  thirty-six  years 
old,  was  admitted  into  the  hospital  on  the  12th  of  February, 
1896.  Five  months  before,  he  had  acquired  syphilis,  for 
which  he  had  received  mercurial  injections. 

Four  months  after  the  infection  a  left-sided  facial 
paralysis  developed,  which  was  preceded  for  four  weeks  by 
severe  pains  in  the  back  part  of  the  head  and  over  the  left 
eye.  The  hearing  in  the  left  ear  was  also  affected.  Two 
weeks  after  his  entrance  into  the  hospital  diplopia  appeared. 

The  patient  was  anaemic  and  poorly  nourished.  Evi- 
dences of  past  syphilis  were  found  in  the  form  of  a  scar 
on  the  glans  penis  and  indolent  swelling  of  the  glands  in 
the  groins. 

The  left  facial  nerve  was  completely  paralyzed,  with 
involvement  of  the  soft  palate  and  the  sense  of  taste.  The 
hearing  in  the  left  ear  was  lost  for  both  air  and  bone  con- 


.     SYPHILIS  OF  THE  BASE  OF  THE  BRAIN  123 

duction,  with  normal  otoscopic  findings.  The  patient  also 
complained  of  a  roaring  noise  in  the  left  ear. 

Examination  of  the  eyes  showed  a.  left  abducens 
paralysis  and  a  narrowing  of  the  field  of  vision.  On  the 
left  side  of  the  face  the  sense  of  pain  was  not  quite  so  acute 
as  on  the  right. 

The  remainder  of  the  cranial  nerves  were  not  affected. 

There  existed  a  slight  paresis  of  the  right  arm,  with 
increase  of  the  tendon  reflexes.  Increase  of  the  reflexes 
extended  also  to  the  right  leg  without  any  other  evidence  of 
paresis.  The  cranium  was  more  sensitive  as  a  whole  to 
percussion  on  the  left  side. 

Under  large  doses  of  mercury  and  potassium  iodid  all 
headache  disappeared.  In  the  course  of  four  months  the 
abducens  paresis  and  disturbances  of  sensation  of  the  right 
side  of  the  face  went  away,  the  hearing  improved,  the 
paresis  of  the  right  arm  cleared  up,  and  the  paralysis  of 
the  facial,  except  for  a  slight  trace,  disappeared. 

A  case  of  basilar  meningitis  with  involvement  of  the 
facial  and  acoustic  nerve,  with  severe  mental  disturbance: 

On  the  28th  of  November,  1898,  I  saw  in  consultation 
a  young  man  twenty-five  years  old,  who  fifteen  months  be- 
fore had  acquired  syphilis.  The  primary  lesion  was  an 
unusually  extensive  ulceration,  which,  together  with  the 
macular  eruption  which  followed,  healed  slowly  under  in- 
tensive mercurial  treatment.  Six  weeks  after  the  healing 
of  the  primary  lesion,  which  required  about  two  months,  a 
papular  eruption  appeared,  which  again  made  necessary 
mercurial  treatment.  Nine  months  after  the  infection,  and 
while  the  patient  was  still  under  treatment,  a  sarcocele  de- 
veloped, because  of  which  the  patient  again  received  mer- 
curial injections  and  potassium  iodid.  A  month  later  the 
patient  complained  of  a  continual  headache.  Two  weeks 
before  I  saw  him  he  was  seized  suddenly  in  his  office  with 
a  severe  attack  of  dizziness  and  vomiting.  He  went  to  bed 
right  after  this  attack,  and  the  next,  morning  the  left  facial 
was  completely  paralyzed.  The  patient  was  also  dazed  and 
later  somnolent. 

The  somnolence  lasted  six  days,  the  facial  paralysis 


124  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

remaining  unchanged.  There  was  also  complete  loss  of 
hearing  in  the  left  ear. 

The  ophthalmoscopic  examination  was  nonnal.  There 
were  no  irregularities  of  the  pupils  or  paralysis  of  the  eye 
muscles,  also  no  spinal  symptoms. 

The  facial  paralysis,  by  a  marked  reduction  in  the  direct 
and  indirect  excitability  of  the  faradic  current,  was  shown 
to  be  a  peripheral  one,  and  localized  proximally  from  the 
geniculate  ganglion.  The  examination  of  the  ear  revealed 
the  loss  of  hearing  to  be  caused  by  a  paralysis  of  the  eighth 
nerve.  One  week  later  one  could  demonstrate  the  reaction 
of  degeneration  in  the  musculature  of  the  affected  facial. 

The  patient  was  again  treated  with  large  doses  of  mer- 
cury and  potassium  iodid.  A  maniacal  condition  now  ap- 
peared, to  which  soon  were  added  well-developed  delusions 
of  an  erotic  nature.  The  maniacal  excitement  developed 
to  such  an  extent  that  the  patient  had  to  be  transferred  to 
the  unruly  department  of  the  hospital.  Here,  besides  the 
delusions  above  referred  to,  he  had  delusions  of  persecution 
and  thought  some  one  was  putting  poison  in  his  food,  which 
caused  him  to  refuse  his  nourishment. 

Two  weeks  later  he  developed  ideas  of  grandeur,  which 
soon  plainly  took  on  the  character  of  mental  feebleness.  He 
imagined  that  he  had  discovered  the  syphilis  bacillus  and 
wished,  like  Pasteur  and  Koch,  to  earn  thereby  many  mil- 
lions a  year.  For  this  reason  he  demanded  damages  for 
every  day  which  he  was  kept  in  the  hospital  to  the  amount 
of  20,000  marks. 

The  paralysis  of  the  seventh  nerve  remained  entirely 
refractory  to  treatment. 

The  patient  was  now  transferred  to  a  private  asylum. 

A  year  later  I  received  a  letter  from  the  young  man's 
father,  which  stated  that  after  eight  months  in  the  private 
institution  his  son  was  discharged  entirely  cured,  and  has 
since  remained  well.  To  a  letter  to  the  father,  asking  in 
regard  to  the  paralysis  of  the  facial  and  of  the  auditory 
nerves,  I  received  no  answer. 

This  case  is  a  very  important  one.  It  serves  to  prove 
that  not  only  in  old  age  and  in  weakened  individuals,  but 


SYPHILIS  OF  THE  BASE  OF  THE  BRAIN  125 

also  in  young  and  healthy  persons,  the  nervous  system  may 
become  diseased  soon  after  the  infection.  It  shows  further 
that  organic  and  functional  (intoxication)  disease  can  ap- 
pear at  the  same  time,  and  that  energetic  antispecific  treat- 
ment is  not  always  able,  even  in  strong  and  healthy  persons, 
to  protect  the  nervous  system. 

The  following  case  is  a  combination  of  basilar  and  cor- 
tical meningitis  with  optic  neuritis  and  partial  oculomotor, 
abducens,  trochlear,  and  facial  paresis,  with  severe  epilep- 
tic attacks,  in  all  probability  caused  by  disease  of  the  cortex. 

An  eighteen-year-old  boy,  one  and  one-half  years  ago, 
was  infected  extragenitally  on  the  under  lip  with  syphilis. 
A  week  before  he  came  to  the  hospital  he  had  to  give  up 
his  work  as  house-servant  because  of  severe  headache, 
attacks  of  dizziness,  and  a  feeling  of  great  exhaustion.  He 
came  to  the  hospital  because  his  eyesight  began  to  fail. 

He  was  slightly  dazed  on  his  admission  into  the  hospital. 
On  his  neck  traces  of  leucoderma  were  found,  likewise  on 
his  body.  In  addition  to-  these  evidences  of  lues,  there  was 
a  general  indolent  swelling  of  the  glands.  He  complained 
of  severe  headache,  and  especially  of  pain  deep  in  the  orbits 
of  the  eyes.  There  were  choked  discs  on  both  sides,  with 
numerous  hemorrhages  in  the  retinae.  The  skull  was  dif- 
fusely sensitive  to  percussion,  the  power  of  vision  was 
much  diminished. 

Under  antispecific  treatment  of  six  days'  duration  the 
headache  and  boring  pain  in  the  eyes  improved. 

The  mydriatic  pupils  at  the  time  of  his  entrance  into  the 
hospital  reacted  very  slowly  to  light.  Now  this  reaction 
was  entirely  absent;  both  abducens  were  paretic,  the  right 
more  so  than  the  left.  A  slight  paresis  could  also  be  dem- 
onstrated in  the  trochlear  nerve  and  the  right  facial, 
involving  the  muscles  around  the  mouth. 

The  behavior  in  the  reaction  of  the  pupils  showed  a 
striking  change.  In  three  weeks  the  left  pupil  reacted 
plainly,  the  right  pupil  not  at  all  to  light.  Four  weeks  later 
on  both  sides  an  almost  normal  reaction  to  light  was  present, 
and  in  another  week  both  again  were  mydriatic  and.  without 


126  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

reaction  to  light,  and  on  the  very  next  day  both  were 
prompt  in  their  reaction  to  light. 

Under  antispecific  treatment  the  choked  discs  with  the 
extensive  retinal  hemorrhages  disappeared,  but  now  a  spe- 
cific chorioiditis  was  revealed,  which  had  existed  before. 
This  also  slowly  disappeared,  and  there  was  finally  left 
only  a  slight  pallor  of  the  papillae.  The  paralysis  of  the 
eye  muscles  had  cleared  up. 

Four  weeks  after  the  patient's  entrance  into  the  hospital 
he  was  seized  with  severe  epileptic  convulsions,  which, 
came  on  either  after  premonitory  symptoms,  such  as  head- 
ache, dizziness,  paraesthesias  in  the  left  upper  extremity, 
feelings  of  drowsiness  and  fulness,  or  without  these;  also 
convulsions  in  the  bulbi  and  the  left  face  musculature.  The 
head  was  drawn  toward  the  left;  the  entire  body  became 
rigid.  The  attacks,  which  began  while  the  patient  was  con- 
scious, produced  unconsciousness,  which  lasted  several  min- 
utes and  was  followed  by  deep  depression  and  a  transient 
paralysis  of  the  left  upper  extremity  which  lasted  about 
ten  minutes.  A  second  attack  similar  to  the  first  one  in- 
volved, however,  first  the  left  side,  then  both  extremities. 
A  third  attack  from  the  beginning  consisted  of  general  con- 
vulsions. In  a  fourth  attack  there  were  only  clonic  con- 
vulsions in  the  eyelids,  parassthesias,  and  sensation  of  pain 
in  the  left  arm.  The  consciousness  remained  intact. 

In  the  further  course  of  the  disease  the  patient  often 
complained  of  headache  and  dizziness.  After  a  six  months' 
stay  in  the  hospital  he  was  discharged  in  good  condition. 
The  only  objective  symptom  which  remained  was  a  slight 
pallor  of  the  papillae. 

The  patellar  reflexes,  which,  as  the  only  spinal  symptom 
for  a  long  time,  were  entirely  absent,  again  reappeared. 

We  observe  also  in  this  case,  where  the  patient  was  both 
young  and  strong,  and  the  secondary  symptoms  had  been 
thoroughly  treated,  that  symptoms  of  both  basilar  and  cor- 
tical nature  as  well  as  spinal  symptoms  developed  after 
two  years. 

General  Description  of  the  Paralysis  of  the  Eye  Musculature 
in  Nervous  Syphilis. — The  transient  character  of  a  paralysis 


SYPHILIS  OF  THE  BASE  OF  THE  BRAIN  127 

of  the  eye  muscles  cannot  be  said  to  be  characteristic  of  its 
specific  nature,  since  in  such  postsyphilitic  conditions  as 
tabes  dorsalis  one  quite  often  encounters  this  symptom. 

The  Early  Appearance  of  Paralysis. — Paralyses  of  the  eye 
muscles  may  appear  in  the  earlier  stages  of  the  disease. 
Jefferson  reports  a  case  in  which  this  occurred  six  months 
after  the  infection.  Saenger  saw  a  paralysis  of  the  oculo- 
motor nerve  in  the  form  of  ptosis  five  months  after  the 
infection,  also  another  case  with  oculomotor  paralysis  and 
disease  of  the  olfactory  and  trigeminus  five  months  after 
the  infection. 

Wilbrand  and  Saenger  report  a  case  of  paralysis  of  the 
left  oculomotor  twelve  weeks  after  the  infection. 

The  Late  Appearance  of  Paralysis. — However,  paralysis  of 
the  eye  muscles  develops  much  more  frequently  in  the 
later  stages  of  lues,  and  individual  forms,  especially  dis- 
ease of  the  internal  eye  muscles,  are  most  apt  to  occur  long 
after  the  infection  has  taken  place. 

Frequency  of  Oculomotor  Paralysis. — Our  pathological 
studies  have  shown  us  that  the  oculomotor  should  be  the 
most  frequently  affected  of  the  cranial  nerves,  and  our 
clinical  experience  confirms  this. 

Uhthoff  in  his  167  autopsies  (17  of  his  own  and  150 
taken  from  the  literature)  of  cerebral  syphilis  finds  the 
oculomotor  nerve  to  have  been  affected  66  times,  the  abdu- 
cens  29,  and  the  trochlear  6  times.  In  259  cases  of  brain 
syphilis  he  found  the  oculomotor  affected  in  96  cases.  He 
is  convinced  that  unilateral,  isolated,  complete,  and  partial 
oculomotor  paralysis  is  much  more  frequent  in  lues  than  in 
any  other  disease. 

Fournier  calls  the  paralysis  of  the  third  nerve  the  eye 
paralysis  par  excellence. 

Alexander  reports  in  146  cases  of  specific  paralysis  of 
the  motor  ocular  nerves  involvement  of  the  oculomotor  in 
65  per  cent.,  the  abducens  in  33.5  per  cent.,  and  the  troch- 
lear in  1.5  per  cent. 

Uhthoff  believes  that  bilateral  involvement  of  the 
oculomotor  is  almost  as  frequent  as  unilateral.  In  100 
clinical  cases  of  brain  lues  the  oculomotor  was  affected  in 


128  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

36.  From  these  36  there  was  bilateral  involvement  in  15 
cases. 

Complete  Paralysis  of  the  Oculomotor  Nerve. — The  com- 
plete paralysis  of  the  oculomotor  nerve, — that  is,  the  in- 
volvement of  all  its  branches,  both  internal  and  external, — 
occurs  far  less  often  than  the  partial  paralysis.  Alexander 
reports  19  cases  in  which  there  was  a  total  paralysis  of  the 
nerve  and  145  cases  in  which  there  was  a  partial. 

Mauther's  Theory  of  Nuclear  and  Peripheral  Paralysis. — 
Mauther  has  propounded  the  theory  that  the  oculomotor 
trunk  is  affected  when  all  its  branches  are  interfered  with 
in  their  functioning,  and,  vice  versa,  when  only  a  partial 
paralysis  exists  a  particular  nuclear  group  is  involved. 
This  theory  fits  in  very  well  with  the  anatomical  relations 
which  have  been  schematically  presented  of  the  various 
nuclear  groups.  It  also  helps  to  explain  the  frequent  cases 
of  paralysis  of  isolated  muscles,  especially  the  levator  pal- 
pebrae  superior.  Clinical  and  pathological  observations  at 
the  present  time,  however,  will  not  justify  us  in  this  view. 

Uhthoff  has  conclusively  proven,  both  from  his  own  and 
other  cases,  that  where  apparently  the  disease  of  the  oculo- 
motor trunk  was  uniform  a  disturbance  of  function  of  only 
certain  muscles  may  be  produced,  so  that  in  a  genuine 
basilar  affection  it  may  happen  that  only  the  external  or 
the  internal  muscles  will  be  paralyzed,  and  that  even  where 
both  macroscopic  and  microscopic  disease  of  the  trunk  have 
been  shown,  the  function  can  remain  entirely  intact. 

Perineuritis  of  the  Individual  Intraorbital  Branches  of  the 
Oculomotor. — Siemerling  has  described  such  a  case.  A 
basilar  gummatous  process  had  penetrated  into  the  orbit 
and  then  followed  along  the  branches  of  the  nerve. 

In  the  case  reported  by  Wilbrand  and  Saenger,  where 
there  was  a  paralysis  of  the  left  oculomotor  nerve,  to- 
gether with  an  anaesthesia  in  the  area  supplied  by  all  three 
branches  of  the  fifth  and  a  paralysis  of  the  facial  and 
auditor^7  nerves,  the  lesion  must  have  been  a  gummatous 
neuritis. 

Secondary  Disease  of  the  Oculomotor  in  the  Orbital  Fissure. 
— Secondary  disease  of  the  nerve  in  the  orbital  fissure  is, 


SYPHILIS  OF  THE  BASE  OF  THE  BRAIN  129 

_ 
however,  more  frequent,  since  here  the  optic,  oculomotor, 

trochlear,  abducens,  and  first  branch  of  the  fifth  lie  close 
to  one  another  in  a,  narrow  opening,  whose  bony  walls,  as 
well  as  the  periorbital  tissue  which  fills  in  the  space,  are 
quite  susceptible  to  specific  disease.  We  then  have  a  char- 
acteristic clinical  picture  of  amaurosis,  paralysis  of  both 
the  external  and  internal  ocular  muscles,  anaesthesia  in  the 
area  of  the  first  branch  of  the  fifth,  pain  deep  in  the  orbit, 
oedema  of  the  upper  eyelid,  and  slight  exophthalmus. 

Disease  of  the  Oculomotor  in  the  Nucleus  and  Root  Area. — 
Gummatous  meningitis  is  the  most  frequent  cause  of  dis- 
ease of  the  third  nerve  on  the  base  of  the  brain.  The  nerve 
is  either  compressed  in  the  inflammatory  process  or  the  in- 
flammation extends  directly  to  the  nerve  itself  in  the  form 
of  neuritis  and  perineuritis  gummosa.  Gummata.  may  also 
develop  in  the  region  of  the  nucleus  and  root  area,  or  these 
neighborhoods  may  be  damaged  by  lesions  in  the  pons  and 
crura.  The  nucleus  may  be  entirely  diseased  and  still  only 
a  part  of  its  fibres  may  be  affected.  Ophthalmoplegia  ex- 
terna,  the  paralysis  of  all  the  voluntary  eye  muscles  sup- 
plied by  the  third  nerve,  is  most  commonly  caused  by  dis- 
ease of  the  nerve-trunk. 

Of  the  individual  external  branches,  paralysis  of  the 
branch  which  elevates  the  eyelid,  ptosis  is  by  far  the  most 
common  and  often  the  only  paralysis  of  the  eye  muscles  in 
brain  syphilis.  Lancereaux  says  it  is  almost  pathogno- 
monic  of  lues. 

In  Disease  of  the  Oculomotor  Trunk  Isolated  Paralysis  May 
Occur. — The  isolated  position  of  the  particular  nuclei  which 
furnish  the  nerve  supply  of  the  elevator  muscle  of  the  eye- 
lid would  cause  one  to  suppose  that  this  paralysis  would  be 
nuclear  in  origin.  However,  as  Wilbrand  and  Saenger  have 
pointed  out,  there  has  never  been  any  proof  pathologically 
of  this.  On  the  other  hand,  there  have  been  a  number  of 
cases  reported  of  isolated  specific  ptosis  where  disease  of 
the  nerve-trunk  has  been  found. 

Involvement  of  the  Internal  Oculomotor  Branches. — Among 
the  affections  of  the  internal  branches  of  the  oculomotor 
nerve,  paralysis  of  the  pupils  is  extremely  frequent,  mydri- 


130  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

asis  and  the  loss  of  the  pupil  reactions,  both  to  light  and 
convergence,  and  their  combination  with  paralysis  of 
accommodation  (ophthalmoplegia  interna). 

Pupil  Anomalies  in  Normal  Persons. — In  regard  to  anoma- 
lies of  the  pupils  as  to  size  and  shape  in  normal  persons 
we  are  indebted  to  a  number  of  observers  for  statistics. 
In  14,392  cases  examined,  Uhthoff  found  slight  irregulari- 
ties in  256,  or  in  18  per  cent. ;  Ivanoff  in  134  cases,  in  12,  or 


FIG.  37. — Isolated  ptosis  in  a  syphilitic. 

9  per  cent. ;  Dufour  in  173  cases,  in  8,  or  4.5  per  cent. 
Anomalies  of  function  were  not  discovered  in  any  of  these 
cases. 

Among  those  diseases  which  cause  isolated  anomalies 
of  the  pupils  one  should  consider  arteriosclerosis  first, 
which,  through  disease  of  the  arteries  in  the  nucleus  and 
secondary  softenings,  can  cause  changes  both  in  the  size 
and  function  of  the  pupils. 

Pupil  Anomalies  in  Syphilitics. — Either  unilateral  or  bilat- 
eral myosis  with  loss  of  the  pupil  reaction  to  light  and 
convergence,  accommodation  remaining  intact,  is  quite  a 
frequent  pupil  defect  in  luetics. 

E.  Meyer  in  74  cases,  Bultino  in  70,  and  Mantoux  in  101, 
each  saw  one  case  of  loss  of  the  reaction  of  both  pupils 


SYPHILIS  OF  THE  BASE  OF  THE  BRAIN  131 

to  light  without  any  other  objective  symptoms  on  the  part 
of  the  nervous  system.  These  cases  were  all  in  persons 
who  previously  had  had  syphilis ;  otherwise  they  were  nor- 
mal. The  same  thing  has  also  been  observed  by  me  several 
times.  In  one  case  in  particular  which  received  a  most 
thorough  examination  of  the  nervous  system  no  other 
somatic  sign  could  be  discovered  which  in  the  least  could 
make  me  suspicious  of  beginning  tabes  or  general  paresis. 

Since  then  I  have  seen  isolated  pupil  irregularities  in  a 
woman  twenty-seven  years  old,  in  a  man  thirty  years  old, 
in  a  man  fifty-two  years  old,  in  a  physician  forty-seven  years 
old,  and  in  two  gentlemen,  forty-six  and  forty-eight  years 
old  respectively.  In  these  cases  the  time  of  specific  infec- 
tion varied  from  five  to  fifteen  years. 

There  were  three  cases  of  anisocoria  and  two  cases  of 
double  myosis.  Anomalies  of  the  accommodation  or  in  the 
movements  of  the  bulb  were  not  observed.  I  have  had  the 
opportunity  of  observing  these  cases  over  a  number  of 
years,  varying  from  six  to  twelve,  and  I  wish  to  emphasize 
the  fact  that  other  symptoms  which  would  cause  one  to  be 
suspicious  of  tabes  or  paresis  have  not  appeared. 

The  isolated  loss  of  the  reaction  of  the  pupil  to  light 
and  convergence  in  syphilitics  has  also  been  observed  by 
me  several  times.  Three  such  cases  have  been  under  my 
observation  for  eight,  nine,  and  eleven  years  respectively. 

Anomalies  in  the  Reaction  of  the  Pupils  in  Persons  Who 
Have  Never  Had  Syphilis  Have  Frequently  Been  Observed  by 
Me. — From  a  review  of  the  literature  one  is  apt  to  get  the 
impression  that  isolated  pupil  irregularities  are  positive 
symptoms  of  lues.  It  is  important  to  bring  out  here  that 
this  is  not  always  the  case,  although  such  an  isolated  symp- 
tom should  cause  one  to  be  extremely  suspicious  of  syphilis. 

The  following  are  reports  on  cases  of  this  kind  which 
have  come  under  my  observation : 

1.  Male,  fifty-three  years  old,  alcoholic,  right  eye,  loss 
of  reaction  to  light,  left  reaction  slow,  anisocoria,  and  dis- 
tortion of  the  pupils. 

2.  Male,  fifty-eight  years  old,  alcoholic,  bilateral  myosis 
and  loss  of  the  light  reaction. 


132  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

3.  Male,  twenty-six  years  old,  alcoholic,  right  pupil  my- 
otic,  left  slightly  mydriatic;  right  light  reaction  normal, 
left,  loss  of  the  reaction  to  light. 

4.  Male,  forty-three  years  old,  alcoholic,  anisocoria,  loss 
of  the  light  response  on  both  sides. 

5.  Male,  fifty-three  years  old,  non-alcoholic,  loss  of  both 
the  reaction  to  light  and  convergence  in  both  eyes. 

6.  Male,   forty  years  old,  non-alcoholic,  niyosis,  both 
eyes,  and  loss  of  the  pupil  reflexes  to  light. 

7.  The  wife  of  this  man,  who  was  also  a  non-syphilitic, 
had  a  left  ophthalmoplegia  interna  as  an  isolated  symptom. 

8.  A  maiden  lady,  forty-eight  years  old,  with  slight  men- 
tal depression  without  paretic  or  psychic  stigmata,  had 
myosis  and  loss  of  the  light  reaction  on  both  sides. 

9.  Male,  fifty-two  years  old,  alcoholic,  anisocoria,  both 
pupils  irregular  in  shape,  loss  of  light  reaction  in  both, 
spinal  fluid  lymphocytosis  slightly  positive,  globulin  reac- 
tion negative. 

10.  Male,  forty  years  old,  alcoholic,  light  reaction  lost  in 
both  pupils. 

11.  Male,  forty-four  years  old,  alcoholic,  myosis  and  loss 
of  the  light  reaction  on  the  left  side,  lymphocytosis  and 
globulin  reactions  negative. 

In  seven  of  these  eleven  cases  severe  alcoholism  was 
present;  in  four  no  toxic  agent  could  be  discovered. 

It  is  interesting  to  observe  the  reaction  in  the  spinal 
fluid  in  these  cases  of  isolated  pupil  anomalies  both  with 
and  without  evidence  of  syphilis  in  the  history. 

In  those  cases  where  syphilis  could  be  demonstrated 
there  was  only  one  which  reacted  negatively  in  regard  to 
the  globulin  and  lymphocytosis.  On  the  other  hand,  in  eight 
of  the  eleven  cases  reported  above,  in  which  a  spinal  punc- 
ture was  made,  both  the  reactions  were  negative  in  six 
cases.  In  the  other  two,  one  gave  a  weak  positive  globulin 
reaction  and  a  moderate  lymphocytosis,  the  other  a  slight 
lymphocytosis  and  a  negative  globulin  reaction. 

Uhthoff  in  100  cases  of  cerebral  syphilis  found  loss  of 
the  light  reaction  with  intact  convergence  in  ten  cases,  and 
loss  of  both  the  light  reaction  and  convergence  in  four. 


SYPHILIS  OF  THE  BASE  OF  THE  BRAIN  133 

He  has  called  attention  to  the  wide  difference  in  the 
frequency  of  appearance  of  these  pupil  irregularities  in 
tabes  and  cerebrospinal  lues. 

In  tabes  they  occur  in  from  60  to  90  per  cent,  of  the 
cases,  in  cerebrospinal  lues  in  14  per  cent. 

Mikloszewski  pointed  out,  in  1900,  that  a  variation  in 
the  size  of  the  pupils  cannot  always  be  regarded  as  con- 
clusive evidence  of  organic  disease.  He  has  observed  this 
phenomenon  twice  in  entirely  normal  persons,  twice  in 
functional  neuralgias,  four  times  in  hysteria,  and  once  each 
in  vitium  cordis,  nephritis,  tuberculosis,  and  acute  rheuma- 
tism. He  explains  the  phenomenon  by  the  assumption  of  a 
functional  alteration  in  the  sympathetic  nervous  system. 

Ophthalmoplegia  Interna. — OphthaJmoplegia  interna  (pa- 
ralysis of  the  pupils  and  paralysis  of  accommodation)  is 
stated  by  Alexander  as  the  most  frequent  manifestation  of 
syphilis  on  the  motor  apparatus  of  the  eye. 

This  affection,  in  Alexander's  observations,  always  ap- 
peared as  unilateral.  In  77  such  cases  which  he  has  had  the 
opportunity  of  observing,  in  76  per  cent,  lues  either  as  a 
positive  or  probable  cause  could  be  demonstrated.  Mooren 
discovered  syphilis  in  two-thirds  of  such  cases  examined  by 
him,  while  Uhthoff,  on  the  other  hand,  found  syphilis  in 
only  23.3  per  cent,  of  his  cases. 

In  two  cases,  both  in  women,  I  saw  a  unilateral  oph- 
thalmoplegia  interna  as  an  isolated  symptom  on  the  part 
of  the  nervous  system.  In  one  case  exposure  of  the  face  to 
cold  could  be  ascribed  as  the  cause.  In  the  other  there  was 
no  etiological  factor  apparent.  In  both  cases  my  observa- 
tion extended  over  a  period  of  three  and  four  years  respec- 
tively without  the  development  of  other  symptoms. 

The  cases  of  Alexander  which  recovered  developed  later 
into  tabes.  In  other  cases  the  Ophthalmoplegia  interna 
proved  to  be  a  prodromal  symptom  of  severe  mental  dis- 
turbances, usually  of  paresis. 

A  Precursor  of  Postsyphilitic  Disease. — It  is  extremely  im- 
portant to  remember,  however,  that  generally  Ophthalmo- 
plegia interna,  as  well  as  the  combination  with  it  of  myosis, 
is  a  precursor  of  severe  postsyphilitic  disease  of  the  brain 


134  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

and  spinal  cord,  and  that  it  may  be  present  a  long  time  be- 
fore the  outbreak  of  the  tabes  or  paresis.  In  a  patient  of 
Donath  it  was  five  years  after  the  determination  of  the  loss 
of  the  pupil  reflexes  to  light  before  the  development -of  the 
paresis. 

One  could  regard  such  a  case  as  already  tabes  if  only 
a  single  further  symptom,  such  as  loss  of  the  patellar  re- 
flexes, the  Achilles  tendon  reflexes,  or  the  occasional  appear- 
ance of  lightning  pains  in  the  limbs,  or  disturbances  of 
digestion  of  a  crisis-like  nature,  or  girdle  sensations  around 
the  body,  is  present. 

That  such  cases  are  cases  of  tabes  abortiva  or  tabes  im- 
perfecta  should  be  recognized  by  every  one.  One  must 
certainly  admit  that  cases  of  isolated  loss  of  the  pupil  re- 
flexes may  precede  many  long  years  the  appearance  of  a 
well-developed  tabes,  and  so  it  becomes  easier  to  under- 
stand Mobius  and  the  majority  of  the  French  school  when 
they  place  all  of  these  cases  in  the  category  of  tabes.  I 
have  had  four  experiences  which  have  caused  me  to  be 
exceedingly  cautious  in  this  particular. 

The  first  one  was  in  a  physician  in  the  prime  of  life  who 
as  a  student  had  acquired  lues.  During  the  twelve  years 
that  I  knew  him — how  much  longer  I  do  not  know — he  had 
a  bilateral  myosis  with  loss  of  the  pupil  reaction  to  light 
without  other  symptoms,  when  suddenly  he  developed  a 
severe  tabes  with  ataxia  and  paralysis  of  the  bladder. 

The  second  case  was  also  one  of  a  physician  who  as  a 
student  had  contracted  syphilis.  He  had  for  ten  years  a 
double  mydriasis  with  loss  of  the  pupil  reflexes  to  light. 
During  these  ten  years  his  physical  and  mental  condition 
was  extremely  robust  and  his  activity  extensive.  After  a 
short  period  of  depression,  with  change  in  his  appearance 
and  character,  he  developed  a  galloping  form  of  general 
paresis. 

The  other  cases  were  similar  in  nature. 

In  spite  of  such  cases,  however,  I  am  at  the  present  time, 
because  of  my  own  personal  experience,  inclined  to  the 
opinion  that  cases  of  loss  of  the  pupil  reactions  may  occur 
rarely  as  the  only  symptom  of  an  existing  syphilis  without 


SYPHILIS  OF  THE  BASE  OF  THE  BRAIN  135 

the  further  development  in  later  years  of  tabes  or  paresis. 
Summary  of  Oculomotor  Paralysis  Occurring  in  Syphilis. — 

A  brief  enumeration  of  the  various  paralyses  of  the  oculo- 
motor nerve  occurring  in  cerebral  syphilis  will  not  be  out  of 
place  here.  As  we  have  seen,  one  may  have  a  paralysis  of 
both  the  internal  and  external  branches,  or  the  external 
alone,  or  the  internal  alone.  The  external  muscles  likewise, 
either  isolated  or  in  any  combination,  may  be  paralyzed. 
Of  the  internal  muscles,  only  the  sphincter  and  the  muscles 
of  accommodation,  and  these  again  in  different  combina- 
tions with  the  external  muscles  either  unilateral  or  bilateral, 
may  be  affected.  We  have  further  seen  that  ptosis,  the 
paralysis  of  the  elevator  of  the  upper  lid,  is  the  most  fre- 
quent in  brain  lues. 

One  also  often  sees  in  brain  syphilis  both  complete  and 
incomplete  ophthalmoplegia  externa. 

The  opinion  of  Hutchinson  that  such  a  paralysis  in  the 
majority  of  cases  is  due  to  an  affection  of  the  nucleus  is  not 
correct. 

Autopsies  have  shown,  as  in  disease  of  the  collective  ex- 
ternal branches  of  the  oculomotor  nerve,  that  the  lesions  are 
peripheral. 

Disease  of  the  Abducens. — In  regard  to  the  frequency  of 
disease  of  the  abducens,  Uhthoff  in  17  autopsies  found  that 
the  abducens  was  affected  three  times,  and  in  150  cases 
collected  from  the  literature  27  times.  In  the  27  cases  a 
bilateral  involvement  occurred  only  six  times,  which  is  in 
remarkable  contrast  to  the  frequency  of  the  bilateral  affec- 
tion of  the  oculomotor. 

The  pathological  causes  of  disease  of  the  abducens  are 
affections  of  the  pons  in  the  form  of  a  softening  due  to  spe- 
cific arterial  disease,  syphilitic  new  growths,  basilar  luetic 
disease,  and  even  in  rare  cases  a  neuritis  and  permeuritis 
of  the  nerve  itself. 

If  the  abducens  is  affected  within  the  pons  one  finds 
usually  accompanying  the  disturbance  of  the  sixth  nerve  a 
paralysis  of  the  facial  on  the  same  side  or  a  contralateral 
paralysis  of  the  extremities. 

Involvement  of  the  abducens   on  one  side  without  a 


136  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

crossed  facial  or  extremity  paralysis  is  usually  due  to 
basilar  disease. 

Paralysis  of  the  Trochlear. — Uhthoff  found  in  his  17  autop- 
sies involvement  of  the  trochlear  only  once.  In  this  case 
there  was  a  basilar  gummatous  meningitis.  In  the  150 
cases  taken  from  the  literature  the  trochlear  was  affected 
six  times.  The  reason  for  the  rare  involvement  of  the 
trochlear  lies  in  the  protection  which  it  receives  because  of 
its  location. 

It  is  worthy  of  mention  that  the  trochlear  nerve  has 
never  been  reported  in  an  autopsy  as  affected  alone,  but 
always  in  combination  with  other  cranial  nerves. 

Nystagmus. — Nystagmus  has  been  observed  in  cerebro- 
spinal  lues. 

Uhthoff  in  his  review  of  150  cases  found  it  reported  as 
occurring  in  two. 

The  exceedingly  rare  occurrence  of  this  symptom  in 
specific  nervous  disease  is  in  contrast  to  its  rather  frequent 
occurrence  in  other  cerebrospinal  affections,  as  notably  mul- 
tiple sclerosis. 

Paralysis  of  the  eye  muscles  when  the  treatment  has  not 
been  instituted  early  and  carried  out  in  an  energetic  manner 
is  rather  stubborn  and  in  some  cases  entirely  refractory. 

There  are  cases,  however,  of  paralysis  of  the  eye  muscles 
which  are  transient  in  character.  Nevertheless,  the  slight 
tendency  of  the  paralyses  of  these  muscles  to  be  transient 
in  nature  in  basilar  syphilis  is  an  important  diagnostic  dis- 
tinction when  compared  with  the  fleeting  nature  of  the 
paralysis  which  occurs  as  a  precursor  of,  or  in  the  course 
of,  tabes  dorsalis. 


VII 


SYMPTOMATOLOGY  AND  PROGNOSIS  OF  SPE- 
CIFIC BASILAE  MENINGITIS 

The  Differential  Diagnosis  of  the  Paralysis  of  the  Eye 
Muscles  in  Cerebrospinal  Lues  and  Tabes  Dorsalis. — There  is 
a  similarity  between  the  two  paralyses.  Isolated  paralysis 
may  occur  in  both;  likewise  ptosis  and  pupil  disturbances 
are  especially  frequent,  and  one  observes  in  both  ophthalmo- 
plegia  externa. 

Tabes  Dorsalis. — Foumier  states  as  a  means  of  differen- 
tiation (1)  that  in  tabes  the  paralysis  of  the  oculomotor 
is  only  a  partial  one;  (2)  the  external  paralyses  are  often 
found  in  combination  with  the  Argyll-Robertson  phenom- 
enon (the  loss  of  the  pupil  reaction  to  light,  convergence 
remaining  intact),  and  (3)  the  paralyses  of  the  eye  muscles 
in  tabes  are  more  transient  in  character  and  apt  to  recur. 

These  points  of  differentiation  enumerated  by  Fournier 
are  not  at  the  present  time  considered  of  so  much  value  as 
they  were  once  thought  to  be,  because  we  know  now  that 
all  of  these  conditions  may  occur  in  brain  lues.  The  Argyll- 
Robertson  phenomenon  is,  however,  much  more  common  in 
tabes  (according  to  Uhthoff  occurring  in  from  60  to  90  per 
cent,  of  the  cases). 

Saenger  has  called  attention  to  a  distinction  found  by 
him  in  the  examination  of  ten  cases  of  brain  syphilis.  In 
these  cases  the  pupils  of  the  patients  did  not  at  first  react 
to  light,  but  after  having  them  remain  in  a  dark  room  for 
several  hours  he  was  able  to  obtain  the  light  reaction.  In 
cases  of  tabes  the  stay  in  the  dark  room  did  not  restore  the 
light  reaction.  Saenger  sees,  therefore,  in  this  phenomenon 
a  symptom  of  diagnostic  value  between  tabes  and  brain  lues. 

Treupel  has  reported  a  case  of  tabes  with  an  intermit- 
tent loss  of  the  pupil  reaction  to  light. 

This  rare  condition  has  been  observed  by  me  in  two 
cases. 

137 


138  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

With  a  loss  of  the  light  reaction  on  one  side  and  an  optic 
neuritis  on  the  same  side,  one  may  safely  diagnose  the  case 
as  brain  lues. 

In  regard  to  the  pathological  difference  between  tabes 
and  brain  syphilis,  especially  since  the  comprehensive 
labors  of  Siemerling  and  Bodeker,  it  has  been  demonstrated 
that  in  tabes  the  lesion  consists  frequently  in  a  primary 
nuclear  atrophy  along  with  a  primary  peripheral  neuritis. 

The  assumption  that  the  paralysis  of  the  eye  muscles  in 
tabes  is  produced  through  the  trophic  influence  of  the 
sympathetic  has  not  been  proven. 

Siemerling  and  Bodeker  explain  the  transient  nature 
of  these  paralyses  on  the  theory  that  the  disintegrated 
Nissl  bodies  are  capable  of  restitution,  and  that  in  the 
nuclear  atrophy  of  tabes  we  encounter  a,  stage-like  process 
of  cell  deterioration. 

Progressive  Ophthalmoplegia. — In  cases  of  progressive 
ophthalmoplegia  one  must  also  take  into  consideration  the 
possibility  of  bulbar  paralysis  and  disease  of  the  anterior 
horns  of  the  spinal  cord. 

One  may  likewise  encounter  combinations  here,  as  an 
interesting  case  reported  by  Sachs  shows.  In  this  case, 
in  addition  to  an  ophthalmoplegia  and  symptoms  of  an 
atypical  tabes,  there  were  evidences  of  an  affection  of  the 
anterior  horns. 

Brain  Tumor  on  the  Base. — The  differential  diagnosis  be- 
tween a  specific  basilar  meningitis  and  a  brain  tumor  of  the 
base  can  also  present  difficulties.  The  tumors  which  de- 
velop on  the  base  of  the  frontal  lobes  or  in  the  crura,  through 
either  direct  involvement  or  pressure  on  the  basal  nerves, 
may  produce  symptoms  similar  to  those  occurring  in  spe- 
cific meningitis. 

Tumor  of  the  brain  base  of  five  years'  duration, 
paralysis  of  the  eye  muscles  and  disease  of  the  optic  nerve. 
Remissions  and  variations  in  intensity  of  the  symptoms. 
Suspicion  of  lues  in  the  personal  history: 

A  woman,  thirty-six  years  old,  seen  first  by  me  December 
18,  1895,  previous  to  her  present  illness  had  always  been 
healthy.  During  the  past  five  weeks  she  had  suffered  with 


PROGNOSIS  OF  SPECIFIC  BASILAR  MENINGITIS   139 

severe  headache  and  occasional  vomiting.  Soon  after  the 
beginning  of  the  headache  she  complained  of  double  vision. 
Objectively  there  was  found  a  left  abducens  paralysis. 
There  was  also  tenderness  to  percussion  on  the  left  side  of 
the  cranium,  especially  the  left  frontal  region. 

As  a  result  of  two  months  of  mixed  treatment  all  the 
symptoms  disappeared.  The  patient  remained  free  from 
symptoms  for  a  period  of  five  months,  when  the  headache 
and  vomiting  reappeared.  Objectively  there  were  found  at 
this  time,  again,  a  left  abducens  paralysis,  paresis  of  the 
left  rectus  internus  and  the  left  levator  palpebrae  superioris 
muscle,  also  a  slight  optic  neuritis  on  both  sides. 

As  a  result  of  further  antispecific  treatment  the  head- 
ache and  paralysis  of  the  ocular  muscles  disappeared. 
However,  several  weeks  later  both  the  headache  and  the 
eye  paralysis  returned,  and  this  time  did  not  respond  to 
the  treatment.  In  the  summer  of  1898  the  patient  took  two 
months'  treatment  of  hydrotherapy  and  electricity  in  the 
Eppendorf  Hospital  and  appeared  to  have  been  greatly 
benefited  temporarily  thereby. 

The  neuritis  optica  had  become  more  marked  and  bore 
more  the  character  of  choked  disc.  In  the  fall  of  1899  some 
new  symptoms  appeared:  parasthesias  and  pressing  pains 
in  the  left  side  of  the  face,  attacks  of  trismus,  and  dimin- 
ished sense  of  smell  on  the  left  side.  Suspicion  of  a  slow- 
growing  tumor  on  the  base  of  the  brain  was  now  expressed. 

The  condition  of  choked  disc  had  passed  into  a  partial 
atrophy.  The  variation  in  the  power  of  vision  of  the 
patient  from  day  to  day  was  striking.  On  some  days  she 
was  entirely  blind  in  her  left  eye ;  on  others  sometimes  for 
an  hour  or  two;  sometimes  the  entire  day  she  was  able  to 
count  fingers  and  distinguish  between  persons  with  it. 

In  March,  1900,  she  showed  for  the  first  time  a  protru- 
sion of  the  left  eye,  and  began  to  have  epileptiform  attacks, 
both  with  and  without  loss  of  consciousness.  In  two  months 
from  this  time  the  patient  died. 

At  the  postmortem  the  dura  over  both  frontal  lobes  was 
adherent.  The  lamina  cribrosa  of  both  ethmoids  was 
destroyed  and  filled  in  with  a  tumorous  growth.  The  lower 


140  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

half  of  the  left  frontal  lobe  was  occupied  by  a  tumor.  The 
frontal  lobes  inferiorly  were  grown  together  and  an  exten- 
sion of  the  tumorous  growth  could  be  demonstrated  on  the 
lower  surface  of  the  right  frontal  lobe.  The  tumor  was  a 
fibrosarcoma. 

In  this  case  the  onset  with  headache  and  vomiting,  the 
suspicion  of  a  previous  syphilitic  infection,  the  abducens 
paralysis  and  partial  paresis  of  the  oculomotor  nerve,  the 
variation  in  the  ocular  paralyses  and  the  visual  disturb- 
ances, and  the  apparent  improvement  produced  by  anti- 
specific  treatment  were  responsible  in  the  beginning  for  the 
diagnosis  of  syphilitic  basilar  meningitis.  At  the  present 
time  the  four  reactions  would  clear  up  the  diagnosis  in  such 
a  case. 

Meningitis  Cysticercus. — Meningitis  cysticercus  may  also 
present  a  clinical  picture  resembling  basilar  meningitis.  In 
this  form  of  meningitis  the  symptoms  are  subject  to  even 
a  greater  variation  than  in  the  specific  form;  the  course 
is  also  more  acute. 

Multiple  Sclerosis. — In  multiple  sclerosis,  in  addition  to 
the  perhaps  too  much  emphasized  nystagmus,  one  also  finds 
external  and,  in  rare  cases,  internal  paralysis  of  the  ocular 
muscles. 

The  ocular  paralyses  in  multiple  sclerosis  are  also  apt 
to  be  transient  in  nature. 

Disease  of  the  optic  nerve  often  occurs  in  multiple  scle- 
rosis. An  affection  of  the  optic  nerve  may  occur  in  this 
disease  before  any  symptoms  on  the  part  of  the  eyes  have 
appeared.  It  is  apt  to  be  more  transient  in  character  than 
in  syphilis,  and  causes  less  often  total  blindness. 

Since  patients  who  have  previously  been  infected  with 
syphilis  may  have  multiple  sclerosis,  and,  on  the  other  hand, 
cases  have  been  described  in  which  cerebrospinal  sclerosis 
symptomatically  resembled  cerebrospinal  lues,  one  can 
easily  understand  how  under  certain  circumstances  a  dif- 
ferential diagnosis  between  the  two  diseases  may  be  ex- 
tremely difficult. 

In  the  case  of  a  man,  forty- two  years  old,  who  pre- 
viously had  contracted  lues,  because  of  a  sudden  paresis  of 


PROGNOSIS  OF  SPECIFIC  BASILAR  MENINGITIS    141 

the  facial  and  paralysis  of  the  eye  muscles,  which  in  the 
course  of  two  weeks  was  followed  by  a  spastic  paresis  of  the 
lower  extremities,  both  Eisenlohr  and  myself  diagnosed 
cerebrospinal  syphilis.  Only  when  antispecific  therapy  pro- 
duced no  improvement,  and  in  the  further  course  of  the 
case  ataxic  paresis  of  the  upper  extremities,  along  with 
nystagmus,  developed,  did  it  become  clear  that  the  case 
was  one  of  multiple  sclerosis. 

Cerebral  Hemorrhages. — Cerebral  hemorrhages  can  also 
cause  isolated  paralysis  of  the  eye  muscles.  This  is  easily 
explainable  from  the  nature  of  the  blood  supply.  According 
to  Shiamura's  schematic  representation  the  arterial  region 
lying  along  the  median  line,  in  the  neighborhood  of  the 
nucleus  of  the  third  nerve,  composed  of  branches  given  off 
from  the  posterior  communicating  arteries,  can  be  regarded 
as  an  isolated  one,  and  does  not  communicate  with  the 
arterial  system  which  supplies  the  cerebral  peduncles  and 
the  tegmentum.  This  medianly-situated  arterial  region 
supplies  the  blood  for  the  oculomotor  and  red  nuclei. 

Head  Injuries. — The  nerves  of  the  eye  muscles  may  also 
suffer  damage  from  head  injuries. 

Differential  diagnostic  difficulties  will  not  occur  in  most 
of  these  cases,  because  of  the  existing  etiological  factor. 
If  the  patient,  however,  has  had  syphilis,  and  the  physician 
does  not  discover  the  ocular  palsy  until  some  time  has 
elapsed  after  the  accident,  he  may  then  be  in  doubt  as  to 
whether  he  should  attribute  the  condition  to  trauma  or  to 
syphilis. 

Tubercular  and  Epidemic  Meningitis. — Tubercular  basilar 
meningitis  may  be  distinguished  from  the  luetic  variety  by 
the  more  acute  course  of  the  former  and  the  presence  of 
fever,  which  is  probably  never  absent. 

The  same  is  true  of  cerebrospinal  meningitis  of  the  epi- 
demic type. 

Relapsing  Oculomotor  Paralysis. — Oculomotor  paralysis  of 
the  recurring  type  has  nothing  to  do  with  syphilis.  What 
its  origin  is,  at  the  present  time  we  do  not  know.  Cases 
of  this  affection  occur  not  infrequently  with  symptoms  of 
meningeal  irritation,  headache,  stiffness  of  the  neck,  and 


142  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

vomiting.  If  syphilis  is  present  in  the  history,  before  the 
disease  has  completed  its  course,  naturally  the  diagnosis 
will  remain  in  doubt. 

The  Trigeminal  Nerve. — The  trigeminus  nerve  lies  on  the 
base  of  the  brain  between  the  trochlear  and  abducens  nerves. 
The  fifth  nerve  may  in  a  specific  basilar  meningitis  be  either 
involved  alone  or  in  combination  with  other  cranial  nerves. 
A  consideration  of  the  anatomy  of  this  nerve  shows  us  that 
not  only  is  its  nucleus  widely  distributed,  but  also  its 
branches  ramify  extensively  through  the  foramina,  bony 
sinuses,  and  fissures  of  the  cranial  bone.  Specific  proc- 
esses on  the  -bony  edges  of  the  orbit  and  in  the  orbit  itself 
are,  of  course,  liable  to  involve  the  first  branch  of  the 
nerve.  Disease  of  the  second  branch  is  often  associated 
with  the  affections  of  the  nasal  cavities  and  the  antrum. 
Likewise  a  narrowing  of  the  foramen  ovale  on  the  base  of 
the  brain  and  disease  of  the  lower  jaw  would  involve  the 
third  branch.  In  the  interior  of  the  skull,  disease  of  the 
middle  cranial  fossa  may  cause  a  disease  of  some  or  all 
of  the  branches  of  the  nerve.  In  the  region  of  the  nucleus 
one  encounters  all  the  processes  which  have  been  spoken  of 
in  relation  to  the  ocular  paralyses,  hemorrhages,  areas  of 
softening,  sclerosis,  and  tumors. 

One  sees  not  infrequently  isolated-appearing  neuralgias 
in  the  first  and  second  branches  of  the  fifth,  both  in  the 
early  and  late  stages  of  syphilis. 

A  jurist,  who  two  years  before  coming  to  me  had  ac- 
quired syphilis,  was  seized  with  severe  neuralgic  pains  in 
the  first  branch  of  the  trigeminus.  After  the  customary 
medication,  combined  with  electricity,  had  failed  to  relieve, 
antispecific  treatment  was  administered  and  the  neuralgia 
quickly  disappeared.  This  patient  about  every  two  years 
has  a  return  of  his  neuralgia,  which  always  promptly  disap- 
pears under  antispecific  therapy. 

This  case  brings  up  the  question  which  has  often  been 
brought  up  in  regard  to  cases  of  isolated  facial  paralysis 
in  the  early  stages  of  syphilis,  as  to  whether  the  lues  pre- 
disposes to  facial  neuralgia,  or  whether  a  small  localized 
process  on  the  brain  base  exists.  The  frequency  of  appear- 


PROGNOSIS  OF  SPECIFIC  BASILAR  MENINGITIS    143 

ance  of  both  these  conditions  without  any  of  the  accom- 
panying symptoms  of  a  meningitis  speaks  in  favor  of  the 
first  assumption. 

Alexander  reports  two  cases  in  which  facial  neuralgia 
was  the  only  symptom  of  syphilis.  Uhthoff  in  37  autopsies 
found  the  fifth  nerve  affected  alone  four  times. 

Alexander  has  often  observed  involvement  of  the  tri- 
geminal  together  with  the  optic,  facial,  abducens,  and 
trochlear,  and  in  four  cases  he  has  seen  a  keratitis  neuro- 
paralytica  appear  as  the  result  of  lues. 

According  to  Uhthoff,  the  nerves  most  often  affected 
along  with  the  fifth  occur  in  point  of  frequency  in  the  fol- 
lowing order:  optic,  facial,  oculomotor,  abducens,  acoustic, 
olfactory,  and  trochlear. 

Oppenheim  and  Pick  have  both  described  cases  of  de- 
generation of  the  spinal  trigeminal  root.  In  all  of  these 
cases  the  degeneration  was  unilateral  in  contradistinction 
to  the  bilateral  disease  in  tabes.  In  Pick's  case  the  cause 
of  the  degeneration  was  a  destruction  of  the  trigeminal  re- 
gion by  a  gummatous  growth.  In  the  case  of  Oppenheim 
and  Pick  the  degeneration  was  secondary,  due  to  disease  of 
the  Gasserian  ganglion. 

Bumpf  has  been  unable  to  find  in  the  literature  any  case 
in  which  the  sense  of  taste  was  lost  as  a  consequence  of 
syphilis. 

He  himself  reports  one  case  in  which  the  sense  of  taste 
on  the  left  side  of  the  tip  of  the  tongue  was  diminished. 
Under  antispecific  therapy  this  disappeared,  only  to  return 
later.  Two  years  afterward  the  patient  developed  paresis. 

We  will  again  quote  from  Uhthoff  concerning  the  fre- 
quency of  the  involvement  of  the  fifth  nerve  in  syphilis. 
He  found  in  17  of  his  own  autopsies  disease  of  the  tri- 
geminus  three  times,  and  in  150  cases  taken  from  the  litera- 
ture, 22  times.  There  was  involvement  of  the  nerve  on 
both  sides  only  once. 

Involvement  of  the  Facial  Nerve. — As  in  the  case  of  the 
eye  muscles,  the  facial  nerve,  which  runs  a  long  and  devious 
course,  may  be  affected  either  in  its  nucleus,  roots,  or  trunk. 
What  has  been  said  in  regard  to  the  oculomotor  nerve,  that 


144  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

the  gummatous  growths  may  advance  along  the  roots  up 
into  the  nucleus,  is  also  true  of  the  facial. 

I  have  never  been  able  to  find  a  case  of  peripheral  facial 
paralysis,  due  to  Heubner's  arteritis,  in  the  trunk  of  the 
nerve,  although  one  must  admit  the  possibility. 

In  the  facial,  the  electrical  examination,  in  contrast  to 
the  ocular  paralysis,  can  be  utilized  to  differentiate  between 
the  paralyses  which  are  caused  by  lesions  in  the  nucleus 
and  below  it  and  those  which  are  supranuclear  and  cortical. 

The  peripheral  facial  paralysis,  when  it  occurs  in  com- 
bination with  other  paralyses,  whether  they  are  from  the 
cranial  nerves  or  of  the  extremities,  is  found  much  less 
often  than  the  pontine  type.  As  a  matter  of  fact,  one 
frequently  sees  in  brain  syphilis  facial  paralysis  caused 
either  by  areas  of  softening  in  the  pons,  which  is  produced 
by  arteritic  disease  in  the  basilar  artery,  or  by  gummatous 
infiltration,  combined  with  an  alternating  paralysis  of  the 
extremities. 

Not  infrequently  one  encounters  an  isolated  facial 
paralysis  in  secondary  syphilis.  I  have  observed  eight  such 
cases,  cases  which  did  not  differ  apparently  in  any  way 
from  the  ordinary  peripheral  form.  In  these  cases  it  does 
not  seem  that  we  have  to  deal  with  the  ordinary  specific 
basilar  meningitis,  because  the  other  cranial  nerves  are  not 
affected,  and  there  are  no  accompanying  general  symptoms. 
Furthermore,  the  paralysis  runs  a  course  which  is  not  in- 
fluenced by  antispecific  therapy.  We  may  assume  in  these 
cases  that  they  are  simply  produced  by  the  general  injur- 
ious effect  of  syphilis  in  the  body,  as  a  result  of  which  the 
predisposition  to  rheumatic  disease  of  the  facial  is 
increased. 

Some  observers,  as  Boix  and  Dargaud,  take  an  opposite 
view  of  these  cases  and  regard  them  in  every  instance  as 
due  either  to  gummatous  meningitis  or  specific  periosteal 
disease. 

Bernhardt,  Groldflam,  Hatschek,  Gowers,  and  Hoffmann 
believe  that  Boix's  opinion  does  not  hold  good  for  all  cases, 
but  they  also  recognize  that  an  increased  predisposition  is 
produced  in  the  system  as  a  result  of  syphilis. 


PROGNOSIS  OF  SPECIFIC  BASILAR  MENINGITIS    145 

Bernhardt  in  a  monograph  on  recurring  facial  paralysis, 
published  in  1899,  proves  that  recurring  facial  paralysis 
also  occurs  in  luetics.  I  have  observed  one  case  of  this  type 
of  paralysis  in  a  syphilitic  which  developed  twice  after  a 
slight  rheumatic  attack,  the  first  time  without  electrical 
changes,  the  second  time  with  a  partial  R.  D.  Other  nerves 
were  not  affected. 

Both  times  the  paralysis  disappeared  without  antispe- 
cific  treatment. 

Transient  facial  paralyses,  peripheral  in  nature,  are  also 
observed  in  the  initial  stages  of  tabes.  In  such  cases  occur- 
ring in  luetics  one  should  always  keep  in  mind  the  possi- 
bility of  disease  of  the  posterior  columns.  Recently,  iso- 
lated cases  of  facial  paralysis  have  been  given  importance 
as  often  occurring  as  the  so-called  nervous  relapses  (neuro- 
recidive)  after  salvarsan  administration.  Lumbar  punc- 
ture shows  in  many  cases  that  the  three  reactions  are  posi- 
tive. In  a  recent  case  I  found  this  to  be  true.  Such  obser- 
vations prove  what  one  formerly  could  only  regard  as  prob- 
able, that  such  peripheral  facial  paralyses  are  actually 
syphilitic  in  nature. 

Disease  of  the  Eighth  Nerve. — In  1903  Quincke  reported 
two  cases  in  which  the  eighth  nerves  were  affected  in  com- 
mon with  the  seventh.  In  the  first  case,  in  addition  to  the 
involvement  of  both  auditory  and  both  facials,  the  abducens 
was  affected.  Recovery  occurred  under  antispecific  treat- 
ment. In  the  other  case  the  auditory  symptoms  predom- 
inated. This  case  came  to  autopsy.  Here  the  deafness  of 
the  patient  was  found  to  be  due  to  degeneration  of  both 
auditory  nerves,  and  the  facial  paralysis  on  the  right  side 
to  degeneration  of  the  facial  nerve. 

Oppenheim  complains  that  in  the  cases  reported  in  the 
literature  one  frequently  finds  no  clinical  differential  diag- 
nosis made  between  an  affection  of  the  auditory  nerve  and 
the  sound-conducting  apparatus.  In  the  case  reported 
above,  the  diagnosis  "nervous  deafness"  was  made  because 
the  sense  of  hearing  was  decreased  for  moderate  tones, 
normal  for  high  tones,  and  there  was  evidence  of  severe 
functional  exhaustion. 

10 


146  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

Rosenstein  has  made  a  comprehensive  review  of  the 
cases  of  disease  of  the  auditory  nerve  due  to  syphilis.  It 
appears  from  Eosenstein's  studies  that  specific  affection 
of  the  eighth  nerve  is  much  more  frequent  than  has  hitherto 
been  supposed.  The  function  of  the  nerve  has  not  been 
examined  carefully  enough. 

We  know  now  that  specific  neuritis  of  the  auditory  nerve 
may  often  be  slight  in  character  and  sometimes  present  no 
subjective  disturbance  whatever.  Failure  to  examine  thor- 
oughly the  other  cranial  nerves  is  often  responsible  for  the 
failure  of  observing  an  affection  of  the  eighth  nerve.  This 
has  especially  been  shown  to  be  true  since  the  era  of  nervous 
relapses  (neurorecidive).  We  know  now,  since  the  eighth 
nerve  is  being  more  carefully  examined,  that  it  is  affected 
in  the  early  stages  of  lues  much  more  frequently  than  it  was 
formerly  thought  to  be.  On  the  other  hand,  the  mistake  is 
sometimes  made  in  luetics  in  assuming  disease  of  the  audi- 
tory nerve,  when  in  reality  middle-ear  disease  exists. 

The  auditory  nerve  is  more  frequently  affected  in  syph- 
ilis than  the  olfactory.  Frequent  cause  of  its  disease  is  by 
the  extension  of  specific  processes  from  the  petrous  portion 
of  the  temporal  bone  or  through  compression,  as  a  result 
of  periostea!  narrowing  in  the  inner-ear  passages.  The 
most  frequent  cause,  however,  is  basilar  gummatous  menin- 
gitis. 

The  nerve  is  susceptible  to  the  same  degenerative  proc- 
esses in  its  nucleus  and  trunk  as  the  facial.  The  nerve- 
endings  in  the  labyrinth  are  also  sometimes  diseased,  and 
not  infrequently  in  the  early  stages  of  lues. 

Kreiblich  says  that  in  his  experience  the  tendency  has 
been  usually  in  the  early  stages  of  syphilis  to  conclude  that 
the  quickly  appearing  difficulty  in  hearing  was  not  due  to 
disease  of  the  nerve,  but  rather  to  refer  it  to  some  disturb- 
ance in  the  sound-conducting  apparatus,  to  a  transmitted 
inflammation  from  the  throat  and  inflammation  of  the  mid- 
dle ear.  He  has  observed  four  cases  in  one  year  of  disease 
of  the  labyrinth,  and  all  of  them  occurred  in  the  early  stages 
of  lues,  three  or  four  months  after  the  infection,  partly  due 
to  a  purely  cochlear  affection  and  partly  as  a  result  of 


PROGNOSIS  OF  SPECIFIC  BASILAR  MENINGITIS    147 

combined  disease   of  the   vestibule   and   the   semicircular 
canals. 

The  disturbances  in  hearing  consist  in  a  loss  of  the  high 
tones,  while  deep  tones  are  heard  as  disagreeable  sounds. 
Disease  of  the  vestibule  and  semicircular  canals  manifests 
itself  in  dizziness,  nausea,  inclination  to  vomit,  accompanied 
at  the  same  time  by  subjective  noises  and  diminished 
hearing. 

In  one  of  my  cases,  a  young  man  twenty-eight  years  old, 
who  had  contracted  a  lip  chancre  and  during  the  following 
year  and  a  half  took  three  courses  of  inunctions,  soon 
after  the  completion  of  the  third  inunction  course  there 
developed  both  subjective  and  objective  disturbances  of  the 
above-described  nature  in  the  right  ear,  along  with  severe 
headache.  The  headache  disappeared  after  the  administra- 
tion of  potassium  iodid,  while  the  subjective  ear  noises 
continued.  There  was  found  on  the  right  side  a  diminished 
capacity  for  the  perception  of  high  tones,  with  a  subjective 
painful  sensitiveness  to  these  tones.  Deep  tones  were  heard 
normally  straight  out  from  the  ear,  but  to  the  left  were 
intensified;  whispered  speech  was  normal  in  the  left  ear, 
in  the  right  heard  only  when  spoken  directly  into  the  ear. 
A  diagnosis  of  labyrinthine  disease  was  made.  In  spite  of 
large  doses  of  potassium  iodid  in  the  next  few  weeks,  an 
abducens  paralysis  developed,  with  such  intense  dizziness 
that  walking  was  almost  impossible. 

Another  inunction  course  with  sweat  baths  and  large 
doses  of  potassium  iodid  brought  about  recovery  in  the 
course  of  six  weeks.  The  patient  has  remained  well  up  to 
the  present  time,  a  period  of  four  years. 

Meniere's  Symptom-complex. — One  finds  in  the  various 
monographs  on  the  subject  of  brain  syphilis  that  Meniere's 
symptom-complex  may  also  occur  as  the  result  of  cerebral 
lues.  One  may  assume  syphilitic  disease  of  the  bones  in  the 
labyrinth  as  the  etiological  factor.  However,  so  far  as  I 
know,  there  has  been  no  pathological  confirmation  of  this 
assumption. 

Glossopharyngeal  Nerve. — Disturbances  of  the  glosso- 
pharyngeal,  which  supplies  the  posterior  third  of  the 


148  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

tongue,  the  mucous  membrane  of  the  soft  palate,  and  the 
palatine  arch  with  taste,  furnishes  sensation  to  the  tonsils, 
the  posterior  pharynx,  the  mucous  membrane  of  the  tym- 
panic cavity  and  the  cells  of  the  mastoid  process,  and  is 
concerned  in  the  act  of  swallowing,  may  be  manifold  in 
character. 

Reports  of  isolated  disease  of  this  nerve  have  never 
come  under  my  observation,  but  in  all  the  pathological 
processes  which  involve  the  last  four  cranial  nerves, 
whether  in  the  nuclear  region,  the  root  sphere  inside  and 
outside  of  the  medulla,  it  may  also  be  affected.  One  should 
always  be  on  the  lookout,  however,  when  there  are  taste 
disturbances  in  the  posterior  portion  of  the  tongue  for  sup- 
purative  affections  of  the  middle  ear  or  simple  catarrh  of 
the  tympanic  cavity.  Oppenheim  reports  a  case  in  which 
a  pachymeningitis  through  a  puckering  and  compression  of 
the  medulla  and  the  nerves  coming  out  from  it  produced  an 
acute-developing  aphonia,  a  paralysis  of  swallowing  and 
respiration,  tachycardia,  and  hemiatrophy  of  the  tongue. 

Symptoms  Caused  by  Disease  of  the  Vagus. — These  may  be 
manifested  by  pulse  anomalies,  disturbances  of  respiration, 
and  paralysis  of  the  vocal  cords. 

In  a  case  of  paralysis  of  the  left  recurrent  laryngeal  one 
is  apt  to  think  either  of  an  aneurism  of  the  aorta  or  of  an 
infiltrating  process  in  the  apex  of  the  lung,  both  of  which 
may  be  specific  in  nature. 

Enlarged  mediastinal  glands  may  also  cause  vagus 
symptoms. 

Remak  reports  a  case  in  which  twelve  years  after  the 
syphilitic  infection  a  paralysis  of  the  vocal  cords  and  an 
accessory  paralysis  along  with  increased  frequency  of  the 
pulse  appeared. 

In  this  case  Remak  was  in  doubt  whether  to  attribute 
the  symptoms  as  due  to  a  primary  specific  neuritis  or  a 
secondary  injury  caused  by  a  pachymeningitis  syphilitica 
in  the  region  of  the  anterior  roots  of  the  upper  cervical 
nerves. 

The  Hypoglossal  Nerve. — The  hypoglossal,  in  addition  to 
being  involved  along  with  the  other  bulbar  nerves,  may 


PROGNOSIS  OF  SPECIFIC  BASILAR  MENINGITIS    149 

sometimes  become  affected  alone.  Remak  has  reported 
such  a  case  in  which  he  found  the  hypoglossal  compressed 
in  the  condyloid  foramen  through  gummatous  proliferation. 

According  to  the  observations  of  Ballet,  Raymond,  Koch, 
and  Marie  in  paralysis  of  the  vagus  and  accessory,  in  oph- 
thalmoplegia,  and  in  hemiatrophy  of  the  tongue,  one  should 
thoroughly  examine  the  patient  in  order  to  determine 
whether  or  not  tabes  is  the  underlying  cause. 

Bernhardt  has  reported  a  case  of  hemiparalysis  of  the 
tongue  which  was  caused  by  a  syphilitic  swelling  of  the 
cervical  glands.  Dinkier  has  described  a  case  with  atrophy 
of  the  tongue  which  he  explained  by  a  pressure  neuritis  of 
the  hypoglossal  caused  by  a  syphilitic  periostitis  of  the 
condyloid  foramen. 

Pons  and  Medulla. — Symptoms  originating  from  the  brain 
stem  (the  pons  and  medulla)  may  also  be  caused  by  syphilis. 
Here  naturally  one  encounters  the  same  pathological 
lesions,  such  as  tumors  and  arterial  changes,  with  their 
consequent  terminations  in  aneurisms,  hemorrhages,  throm- 
boses, and  softenings. 

Blood  Supply. — It  has  been  recognized  for  a  long  time 
that  aneurismal  formations  of  the  vertebral  artery  as  a 
result  of  specific  arterial  disease  are  comparatively  fre- 
quent, while  in  the  branches  supplying  the  central  ganglia 
they  seldom  occur.  The  arteries  of  the  posterior  cranial 
fossa  are  not  so  liable  to  specific  disease  as  those  in  the 
middle  cranial  fossa.  However,  one  may  observe  through 
specific  disease  of  the  vertebrals  either  an  acute  or  chronic 
bulbar  paralysis. 

Arterial  symptoms  arising  from  the  cerebellum  are  not 
very  common,  because  the  cerebellum  is  supplied  by  two 
large  arteries,  both  branches  from  the  basilar  and  verte- 
bral arteries,  which  afford  good  opportunities  for  collateral 
circulation. 

The  classical  symptom-complex  for  a  focal  lesion  in  the 
cerebral  peduncles  is,  because  of  the  passage  of  the  oculo- 
motor fibres,  a  contralateral  hemiplegia  and  a  homolateral 
oculomotor  paralysis.  Since  the  fibres  of  the  third  nerve 
are  separated  in  the  peduncles  lesions  here  frequently  cause 


150  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

isolated  paralysis  of  the  ocular  muscles  supplied  by  it. 
Hemiplegia  alternans  superior  is  only  encountered  when 
the  lesion  lies  higher  up  in  the  peduncles  and  involves  the 
optic  tract.  We  find  then,  along  with  the  hemiplegia,  a 
hemianopsia. 

When  the  pons  is  affected  there  occurs  then  in  addition 
to  the  contralateral  hemiplegia  either  a  paralysis  on  the 
same  side  of  the  oculomotor,  abducens,  and  facial  nerves  of 
a  peripheral  character,  if  the  lesion  has  affected  their  re- 
spective nuclei  or  their  outgoing  intrapontine  fibres,  or  a 
paralysis  on  the  same  side  of  the  muscles  of  mastication 
points  to  an  involvement  of  the  motor  trigeminal  nucleus, 
a  facial  hemianaesthesia  to  an  affection  of  the  sensory  tri- 
geminal nucleus  or  of  their  outgoing  fibres. 

Two  cases  of  brain  lues  observed  by  me  presented  the 
clinical  picture  of  tumor  of  the  cerebellar  pontine  angle. 
In  one  of  these  cases  on  one  side  there  was  a  paralysis  of 
the  facial  and  auditory  nerves  and  loss  of  the  corneal 
reflex,  while  at  the  same  time  there  was  still  present  on  the 
body  a  late  secondary  papular  eruption.  In  the  other  case 
there  was,  besides  the  paralysis  of  the  seventh  and  eighth 
nerves,  involvement  of  all  three  branches  of  the  fifth  in 
the  form  of  anaesthesia  and  a  keratitis  neuroparalytica. 

Medulla. — An  affection  of  the  medulla,  under  certain 
circumstances,  can  cause,  through  injury  of  the  auditory 
nucleus,  a  central  disturbance  of  hearing,  and  through  in- 
jury of  the  glossopharyngeal  and  vagus  nuclei  and  their 
fibres  a  disturbance  of  the  circulation,  respiration,  and  diffi- 
culty in  swallowing.  A  paralysis  of  the  accessory  will 
manifest  itself  through  a  paresis  of  the  vocal  cords  and  a 
paralysis  of  the  trapezius  and  sternocleidomastoid  muscles. 
Disease  of  the  hypoglossal  causes  a  disturbance  in  the  motil- 
ity  of  the  tongue. 

As  in  the  ordinary  arteriosclerosis,  so  also  in  specific 
endarteritis,  one  may  encounter  the  clinical  picture  of  acute 
or  subacute  bulbar  paralysis  or  of  poliencephalitis  superior 
(Wernicke),  according  as  the  region  supplied  by  the  basilar 
artery  is  affected  and  thereby  the  nuclear  areas  of  the 
oculomotor,  trochlear,  abducens,  and  facial  suffer,  while 


PROGNOSIS  OF  SPECIFIC  BASILAR  MENINGITIS    151 

in  disease  of  the  vertebral  only  the  areas  of  the  strictly 
bulbar  nerves  are  affected. 

In  every  case  of  disease  of  this  portion  of  the  brain  one 
should  bear  in  mind  the  possibility  of  a  syphilitic  cause  and 
when  any  shadow  of  doubt  exists  to  institute  an  energetic 
and  thorough  antispecific  therapy.  The  application  of  the 
four  reactions  here  will  in  most  cases  dispel  the  doubt. 

Glycosuria  and  Polyuria. — In  brain  syphilis  glycosuria 
now  and  then  occurs. 

When  a  patient  presents  himself  who  complains  of  head- 
ache and  has  sugar  in  his  urine,  among  other  etiological 
factors  one  should  not  forget  to  consider  syphilis. 

The  chief  causes  of  this  symptom-complex  are  head  in- 
juries, brain  tumors,  and  brain  lues,  and  very  often  indeed 
brain  gummata  which  are  not  situated  in  the  medulla  pro- 
duce glycosuria.  In  the  majority  of  the  cases  there  is  a 
basilar  gummatous  meningitis  and  a  localization  of  the 
same  in  the  region  of  the  chiasm. 

One  may  assume  this  to  be  the  case  if,  in  characteristic 
sequence,  paralysis  of  the  cranial  nerves  with  polydipsia, 
and  especially  hemianoptic  disturbances  either  with  or  with- 
out hemiplegia,  develop. 

Brain  tumors  in  which  glycosuria  enters  into  the  symp- 
tomatology are  also  most  often  situated  in  the  chiasm 
region. 

There  is,  however,  a  glycosuria  which  occurs  in  syph- 
ilitic arteritis  without  any  symptoms  of  involvement  of  the 
base  of  the  brain  or  of  the  medulla,  in  which  a  hemiplegia 
is  the  only  clinical  expression  of  the  specific  arterial 
affection. 

Diabetes  Mellitus. — There  is  also  a  diabetes  mellitus  in 
syphilitic  patients  which  occurs  as  an  isolated  symptom. 
One  should  regard  such  cases  in  the  same  category  as  those 
cases  of  isolated  idiopathic  epilepsy  and  primary  optic 
atrophy  in  luetics.  It  is  not  produced  by  the  localization  of 
a  specific  process  on  a  certain  spot,  but  develops  as  the 
result  of  the  indirect  influence  of  syphilis  on  the  system. 

Syphilis  does  not  seem  to  be  common  in  diabetics.  In 
an  analysis  of  692  cases  of  diabetes  mellitus  Eumpf  found 


152  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

a  history  of  syphilis  in  84  cases,  13  per  cent.  He  was  un- 
able, however,  in  any  of  the  cases  to  find  either  directly  or 
indirectly  any  relationship  existing  between  the  syphilis 
and  the  diabetes. 

Naunyn  says  that  it  is  quite  probable  that  syphilis  may 
be  a  cause  of  diabetes  mellitus.  He  finds,  however,  after  a 
careful  review  of  the  literature,  that  lues  seldom  produces 
diabetes,  and  that  still  more  seldom  are  any  beneficial  re- 
sults in  diabetes  obtained  by  antispecific  treatment. 

Manchot  in  his  work  on  this  subject  defines  the  required 
limitations  necessary  in  order  to  establish  a  relationship 
between  syphilis  and  glycosuria.  According  to  this  obser- 
ver, it  must  be  proven  that  syphilis  existed  before  the  ap- 
pearance of  the  sugar.  The  glycosuria  must  appear  at  the 
same  time  as  other  symptoms  of  lues  in  the  system,  and, 
lastly,  it  must  recede  under  antispecific  therapy  along  with 
the  other  symptoms  and  without  the  necessity  of  having 
instituted  a  diabetic  diet. 

The  following  case  fulfils  these  requirements. 

Case  of  diabetes  mellitus  with  an  arteritis  luetica  ap- 
pearing at  the  same  time: 

A  merchant,  thirty-six  years  old,  who  eight  months  be- 
fore had  contracted  syphilis,  at  the  time  I  saw  him  was  in 
a  highly-excited  mental  state.  The  year  before  he  had  been 
examined  for  life  insurance  and  was  then  entirely  normal. 

In  the  hospital,  for  a  period  of  about  two  weeks  he  pre- 
sented the  picture  with  normal  somatic  findings  of  a  mani- 
acal state  and  abnormal  irritability.  At  the  same  time 
he  showed  some  evidences  of  grandiose  ideas.  He  com- 
plained of  headache  and  was  unable  to  sleep.  From  the 
time  of  his  entrance  into  the  hospital  there  was  sugar  in 
the  urine,  in  amount  about  3  per  cent.  Under  large  doses 
of  mercury  and  iodid  the  patient  gradually  became  quiet, 
the  sugar  at  first  remaining  the  same.  However,  after  the 
eighth  week  the  sugar  began  to  disappear,  and  at  the  end 
of  four  months  the  quantity  of  sugar  had  diminished  to  0.3 
per  cent.  At  the  end  of  six  months,  the  patient  in  the 
meantime  having  otherwise  fully  recovered,  the  quantity  of 
sugar  was  0.8  per  cent. 


PROGNOSIS  OF  SPECIFIC  BASILAR  MENINGITIS    153 

Diabetes  Insipidus. — Diabetes  insipidus  may  occur  as  a 
result  of  syphilis,  caused  either  by  a  gummatous  brain 
tumor  or  by  a  basilar  meningitis.  A  typical  case  has  been 
described  by  Buttersack  in  which  the  interpeduncular  re- 
gion was  severely  affected.  The  polydipsia  appeared  first. 
Other  cases  have  been  reported  by  various  observers. 

In  all  these  cases  the  polyuria  developed  either  with  or 
after  other  brain  symptoms,  usually  a  long  time  after  the 
infection.  The  most  frequently  associated  symptoms  were 
hemianopsia  and  internal  and  external  ocular  paralysis. 
In  one  of  my  cases  the  infection  had  been  acquired  thirty 
years  before  the  appearance  of  the  diabetes  insipidus. 
Cases  have  been  reported,  although  rare,  in  which  the  poly- 
uria existed  as  the  only  symptom. 

In  some  of  these  cases  the  diagnosis  of  a  specific  cause 
to  account  for  the  diabetes  insipidus  was  made  from  the 
result  of  the  specific  therapy.  In  the  future  we  will  depend 
upon  the  four  reactions  to  establish  this  relationship.  In 
a  case  reported  by  Staub,  however,  the  paralysis  of  the 
eye  muscles  receded  but  the  polyuria  and  polydipsia  re- 
mained uninfluenced. 

Prognosis  of  Brain  Syphilis. — We  will  discuss  the  prog- 
nosis of  brain  syphilis  in  general,  although  the  various 
forms  present  different  prognoses. 

In  the  beginning  of  my  discussion  of  this  subject  I  wish, 
with  Oppenheim,  to  make  this  statement:  that  brain  syph- 
ilis is  a  serious  organic  brain  disease,  which  frequently 
causes  death,  often  leads  to  a  chronic  state,  but  also  in  not 
a  small  number  of  cases  may  end  in  recovery.  The  prog- 
nosis in  brain  syphilis  is  much  more  favorable  than  in  the 
other  organic  affections,  such  as  brain  tumor,  brain  abscess, 
multiple  sclerosis,  and  paresis.  The  first  two  conditions 
may  in  some  instances  be  cured  by  operative  procedures, 
although  in  tumor  cerebri  the  end  result  is  in  the  majority 
of  cases  only  a  partial  one.  In  brain  abscess  also  the  cases 
are  not  frequent  in  which  the  process  does  not  appear  in 
multiple  form  and  before  surgical  relief  can  be  given  has 
caused  a  secondary  meningitis. 


154  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

In  multiple  sclerosis,  although  the  process  may  advance 
very  slowly  and  exhibit  long  remissions,  nevertheless  we 
know  that  it  is  a  disease  which  ends  in  death.  The  same 
is  true  of  paresis. 

To  obtain  a  numerical  representation  of  the  prognosis 
of  brain  syphilis  is  exceedingly  difficult,  because  the  cases 
in  the  first  place  should  have  been  observed  for  a  long  time, 
and  this  is  only  possible  in  a  comparatively  small  number 
of  cases,  since  the  patients  pass  out  from  under  the  phy- 
sician's control  and  later,  when  they  come  under  other 
observation,  not  infrequently  are  not  recognized  as  brain 
syphilis.  In  my  own  experience  I  have  not  been  able  to 
keep  under  a  sufficiently  long  observation  one- third  of  the 
cases  which  have  been  entered  at  Eppendorf  as  cerebral 
lues. 

Statistics. — In  the  following  statistics  only  cases  of  spe- 
cific meningitis,  gummatous  brain  syphilis,  and  luetic  arter- 
itis  have  been  included. 

Fournier  states  that  a  third  of  his  cases  were  cured, 
about  one-half  improved,  and  one-sixth  of  them  died. 
Rumpf  in  31  cases  reports  that  five  died,  eight  remained 
very  sick,  improvement  occurred  in  six,  and  recovery  in 
twelve.  Hjelmann  and  Walter  Berger  do  not  report  so 
favorable  an  experience.  They  estimate  the  fatal  cases  or 
those  who  are  left  with  severe  disturbances  as  about  half, 
the  cases  which  recover  one-fourth.  In  the  remaining  cases 
improvement  occurs.  In  Hjelmann 's  material  death  oc- 
curred in  one-fourth  of  the  cases  within  six  months  after 
the  appearance  of  the  first  cerebral  symptoms,  in  about  one- 
half  of  the  cases  within  the  first  two  years. 

Naunyn  from  88  of  his  own  cases  and  325  collected  out 
of  the  literature  has  made  a  very  careful  study  of  the 
prognosis  in  brain  lues.  He  has  classified  under  recovery 
only  those  cases  in  which  no  relapse  has  occurred  inside  of 
five  years.  He  reports  27  cases  in  this  category.  I  have 
been  able  to  observe  in  124  cases  only  22  which  have  gone 
over  a  period  of  five  years  without  a  relapse.  There  are 
many  more  cases  of  recovery  since  the  last  attack,  of  two  or 
three  years'  duration,  which  Naunyn  has  proven  from  his 


PROGNOSIS  OF  SPECIFIC  BASILAR  MENINGITIS    155 

320  collected  cases,  that  must  be  excluded  from  his  five-year 
period.  They  relapse  before  the  expiration  of  this  time. 

A  Partial  Recovery. — A  partial  recovery  was  found  by 
Naunyn  in  49  of  his  88  cases  and  in  48  per  cent,  of  his  col- 
lected cases.  Among  his  own  cases  ten  became  worse  and 
five  died.  In  56  cases  of  my  own  which  had  remained  well 
for  over  a  period  of  three  years  I  found  a  partial  recovery 
to  be  by  far  of  more  frequent  occurrence.  There  were  34 
such  cases,  10  cases  grew  worse,  and  12  died. 

Recurrences. — Relapses  occur  very  frequently.  In  56  cases 
observed  by  me  for  a  period  of  three  years,  22  cases  re- 
lapsed once,  eight  cases  relapsed  several  times.  It  is  chiefly 
because  of  this  tendency  that  the  uncertainty  in  our  prog- 
nosis exists.  An  apparently  complete  recovery  for  the 
time  being  does  not  offer  any  guarantee  against  a  return  of 
the  disease.  It  is  this  fact  which  has  given  rise  to  the 
expression,  "Syphilis  does  not  die,  it  only  sleeps." 

Relapses  may  appear  again  under  the  same  form;  for 
instance,  a  second  attack  of  arteritis,  an  exacerbation  of 
the  specific  meningitis,  a  new  gummatous  tumor  may  occur, 
or,  on  the  other  hand  an  arteritis  may  follow  a  meningeal 
affection,  or  vice  versa,  as  the  case  may  be.  The  relapse 
may  appear  soon  after  the  termination  of  the  last  outbreak, 
or  years  may  intervene.  The  interval  may  present  a  condi- 
tion of  full  recovery  or  contain  some  symptoms  remaining 
from  the  last  attack. 

The  prognosis  depends  upon  a  number  of  individual  fac- 
tors which  must  be  considered  separately. 

1.  Age  of  Patient  at  Time  of  Infection. — The  first  of  these 
is  the  age  of  the  patient  at  the  time  the  infection  was  con- 
tracted. According  to  both  Hjelmann  and  Naunyn,  age  up 
to  forty  years  has  no  influence  on  the  prognosis.  Both  of 
these  authorities  agree  with  Ricord  and  Fournier  that  the 
prognosis  becomes  worse  the  older  one  becomes  after  the 
age  of  forty  before  acquiring  the  disease.  My  own  experi- 
ence, however,  does  not  entirely  coincide  with  their  view. 
I  have  seen  now  and  then  in  young  persons  brain  syphilis 
run  a  malignant  course,  and,  on  the  other  hand,  cases  of 
both  the  meningeal  and  arteritic  forms  in  patients  who  had 


150  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

been  infected  in  their  forties  and  fifties,  and  in  one  case  in 
the  sixty-fourth  year,  pursue  benign  courses. 

2.  The  Interval  between  the  Time  of  the  Infection  and  the 
Outbreak  of  the  Brain  Affection. — In  this  regard  Hjelmann 
and  Naunyn  agree.     They  both  state  that  the  interval  up 
to  ten  years  is  without  influence  on  the  prognosis,  but  that 
in  a  longer  interval  the  prognosis  becomes  worse. 

In  comparing  my  own  experience  in  124  cases  I  find, 
in  the  four  cases  of  recovery  which  were  kept  under  obser- 
vation over  five  years,  the  interval  in  two  cases  was  two 
years  and  in  two  cases  three  years.  In  those  which  ended 
in  death  the  interval  in  two  was  four  years,  in  three  cases 
five  years,  and  two  cases  each  9,  10,  12,  14,  15  and  30  years 
respectively.  In  the  relapsing  cases  the  interval  varied 
from  6  to  25  years.  In  more  than  one-half  of  the  cases  the 
interval  was  over  10  years. 

3.  Heredity,  a  Bad  Nervous  Inheritance. — Concerning  this 
factor  Oppenheim  makes  this  statement  that  in  so  far  as 
he  has  been  able  to  observe,  a  bad  nervous  inheritance  is 
without  influence  on  the  prognosis.     My  own  experience 
coincides  with  this. 

4.  Debilitating  Agents.— These  are  doubtless  important 
in  the  prognosis.    It  makes  considerable  difference  whether 
a  robust  individual  is  attacked  by  brain  lues  or  one  who 
either   through   a   tubercular  inheritance,   or  tuberculosis 
itself,  through  chlorosis  and  anaemia,  and  especially  through 
alcohol,  has  been  weakened. 

We  have  seen  previously  in  Tarnowsky's  statistics  of 
100  patients  with  brain  syphilis  that  43  were  chronic  alco- 
holics, that  there  were  also  numerous  cases  of  neurasthenia, 
head  injuries,  and  marked  mental  exhaustion.  Tarnowsky 
puts  the  blame  for  a  bad  prognosis  on  such  factors.  He 
regards  the  prognosis  in  patients  who  are  free  from  any  of 
these  harmful  influences  as  very  favorable.  My  own  experi- 
ence does  not  entirely  harmonize  with  Tarnowsky's  view. 
I  have  had  cases  which  ran  a  malignant  course  where  none 
of  these  factors  were  present. 

Head  Injuries. — The  importance  of  head  injuries  has 
probably  been  overestimated.  Oppenheim  states  that  he 


PROGNOSIS  OF  SPECIFIC  BASILAR  MENINGITIS    157 

has  seen  cases  in  which  relapses  have  appeared  apparently 
as  a  result  of  an  injury  to  the  head.  Personally  I  have 
never  been  able  to  observe  that  a  head  injury  very  often 
produces  brain  syphilis,  or  that  the  affection  when  it  did 
develop  was  inclined  to  be  any  more  severe  than  otherwise. 

5.  Form  and  Degree  of  the  Primary  and  Secondary  Symp- 
toms.— A  question  which  has  been  much  discussed  and  is 
important  is  whether  the  course  of  the  syphilis  from  the 
beginning  of  the  primary  lesion  has  any  effect  upon  the 
prognosis  of  a  later-developing  cerebral  lues. 

In  general  all  authorities  are  of  the  opinion  that  syphilis 
of  the  nervous  system,  and  especially  the  parasyphilitic 
affections,  tabes,  and  paresis,  follow  more  frequently  after 
the  milder  types  of  the  primary  and  secondary  symptoms. 
It  has  often  been  said  that  the  mildness  and  insignificance 
of  these  earlier  symptoms  is  the  cause  of  the  later-develop- 
ing visceral  lues,  because  in  such  apparently  mild  forms  the 
antispecific  therapy  was  not  energetic  enough. 

A  perusal  of  the  literature  on  this  point  is  not  very 
satisfactory,  because  one  finds  only  a  very  few  cases  in 
which  the  previous  symptoms  have  been  mentioned.  How- 
ever, it  is  apparent  that  many  cases  occur  in  which  there 
was  a  mild  primary  lesion  and  some  secondary  symptoms 
which  promptly  responded  to  antispecific  treatment.  On 
the  other  hand,  there  are  enough  cases  where  both  the  pri- 
mary lesion  and  secondary  symptoms  were  severe. 

Fournier  is  of  the  opinion  that  there  is  a  type  of  lues 
("Syphilis  a  virus  nerveaux")  which  is  especially  inclined, 
both  early  and  severely,  to  attack  the  nervous  system.  Just 
as  there  are  cases  of  galloping  syphilis,  cases  in  which 
severe  secondary  and  tertiary  symptoms  follow  each  other 
uninfluenced  by  treatment,  so  also  there  are  cases  of  brain 
syphilis  which  never  become  quiescent.  These  cases  may 
appear,  however,  after  either  light  or  severe  primary  and 
secondary  symptoms. 

6.  Extragenital  Infection. — With  reference  to  an  extra- 
genital  infection,  I  have  never  been  able  to  observe  that  it 
has  ever  had  an  unfavorable  influence  upon  a  later  cerebral 
syphilis. 


158  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

7.  Previous  Treatment. — The  question  as  to  whether  the 
mode  of  the  previous  treatment  has  any  influence  on  the 
course  of  a  later-developing  syphilitic  brain  affection  is 
important. 

Many  authors  are  of  the  opinion  that  an  insufficient 
treatment  of  the  primary  and  secondary  symptoms  is  the 
cause  of  the  following  disease  of  the  nervous  system.  Hjel- 
mann  goes  so  far  as  to  say  that  from  82  to  88  per  cent, 
of  the  cases  of  brain  lues  have  either  had  insufficient  treat- 
ment or  no  treatment  at  all;  that  in  the  majority  of  cases 
thorough  antispecific  treatment  would  have  prevented  the 
outbreak  on  the  part  of  the  nervous  system.  On  the  other 
hand,  the  same  author  says  the  prognosis  of  brain  lues 
is  not  influenced  by  previous  mercurial  treatment. 

The  opinions  of  different  authors  are,  unfortunately, 
extremely  contradictory  on  this  point.  While  Kaposi, 
Rumpf,  Lamy,  and  recently  Oppenheim  consider  that  ear- 
lier thorough  and  energetic  antispecific  treatment  improves 
the  outlook  against  the  development  of  brain  syphilis, 
Mauriac,  Leyden,  and  Herxheimer  deny  that  it  does. 

In  my  personal  experience  I  have  observed  so  many 
cases  of  severe  cerebral  lues  which  have  had  such  careful 
earlier  antispecific  therapy  that  I  am  loath  to  believe  that 
the  manner  or  degree  of  previous  treatment  has  any  in- 
fluence worth  mentioning  on  the  later  development  of  brain 
syphilis.  Recently,  however,  Mattauschek,  from  the  study 
of  a  vast  clinical  material  (officers  of  the  Austrian  army) 
that  had  been  under  control  and  observation  for  many 
years,  has  determined  that  in  syphilis  which  has  been  well 
treated  the  nervous  system  is  affected  only  half  as  often 
as  it  is  after  insufficient  treatment. 

All  observers  agree  that  the  timely  and  efficient  treat- 
ment of  the  first  symptoms  of  brain  lues  which  appear  is 
important,  although  I  have  seen  cases — and  cases  are  re- 
ported by  such  authorities  as  Fournier  and  Gilles  de  la 
Tourette— in  which  the  first  manifestations  of  cerebral  lues 
developed  during  antispecific  treatment,  also  cases  in  which 
the  brain  affection,  in  spite  of  intensive  treatment,  steadily 
continued  to  progress.  Such  cases  are,  however,  the  excep- 


PROGNOSIS  OF  SPECIFIC  BASILAR  MENINGITIS    159 

tion.  One  should  remember  that  syphilitic  processes  react 
best  and  quickest  to  treatment  if  they  are  recent  processes, 
which  have  not  as  yet  caused  regressive  changes,  such  as  are 
found  in  gummata  and  connective-tissue  scars. 

Difference  between  Clinical  and  Anatomical  Recovery. — One 
must  take  into  consideration  the  difference  which  exists 
between  a  recovery  in  the  anatomical  sense  and  a  recovery 
in  the  clinical  sense. 

A  gumma  may  be  said  to  have  healed  anatomically  if 
in  place  of  the  specific  gummatous  tissue,  connective  tissue 
has  been  formed.  A  specific  meningitis  is  cured  if  in  place 
of  the  inflammatory  infiltration  scar  tissue  has  been  sub- 
stituted. In  so  far  as  the  clinical  anomalies  are  concerned, 
however,  it  makes  no  difference  whether  they  are  caused 
by  the  presence  of  specific  tissue  or  by  connective  tissue 
which  supplants  the  nerve  substance.  This  factor  stands 
out  more  prominently,  naturally,  in  a  spinal  cord  affection 
than  it  does  in  a  brain  affection,  and  tends  to  weaken  one's 
assurance  in  the  value  of  the  therapy. 

Prognosis  in  Localized  Meningitis. — Specific  meningitis 
represents  the  most  favorable  type  of  brain  syphilis  in  so 
far  as  the  prognosis  is  concerned. 

Hjelmann  says  that  71  per  cent,  of  the  cases  of  recovery 
belong  to  this  form  of  the  disease,  Naunyn  comes  to  a 
similar  conclusion,  and  Oppenheim  states  that  in  ten  cases 
of  complete  recovery  which  came  under  his  personal  obser- 
vation the  essential  symptoms  in  all  of  them  were  pro- 
duced by  a  basilar  meningitis. 

Diffuse  Meningeal  Infiltration. — The  outlook  in  diffuse 
meningeal  infiltrations  is  far  less  favorable,  because  almost 
always  the  nerve  tissue  is  affected  and  frequently  the 
symptoms  are  of  so  indefinite  a  character  for  a  long  time 
that  proper  treatment  is  not  begun  soon  enough. 

The  cases  of  meningitis  of  the  convexity  have,  as  a  rule, 
a  good  prognosis,  although  the  initial  symptoms  may  be 
of  an  alarming  character,  such  as  partial  or  general  epi- 
leptic attacks. 

Isolated  Gummata. — The  true  gummata,  under  which 
classification  I  do  not  include  the  gummatous  processes  of 


160  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

the  many  meningeal  forms  but  only  the  isolated  tumors, 
frequently  react  well  to  mercury  and  potassium  iodid,  but 
sometimes  they  are  quite  refractory  to  treatment  or  they 
react  up  to  a  certain  point  and  then  remain  stationary. 

In  general  one  can  say  that  a  reaction  of  the  symptoms 
to  the  antispecific  treatment,  where  potassium  iodid  is 
administered,  should  make  itself  manifest  at  the  end  of 
one  week ;  where  mercury  is  used,  at  the  end  of  two  weeks. 
It  doubtless  occasionally  happens  that  the  reaction  takes 
place  in  some  cases  later,  but  after  the  third  week  the  out- 
look for  a  favorable  result  from  the  treatment  becomes 
rapidly  worse  if  no  response  has  been  obtained  before. 

Naunyn  in  155  cases  of  recovery  which  he  has  collected 
found  that  improvement  began  either  at  the  end  of  the 
first  or  second  week  after  the  inception  of  the  treatment. 

Arteritis. — The  cases  of  syphilitic  arteritis  have,  as  a 
rule,  a  serious  prognosis.  The  specific  infiltration  in  the 
walls  of  the  arteries  may,  of  course,  be  reabsorbed  in  a 
Heubner's  arteritis.  The  narrowing  of  the  lumen  will,  how- 
ever, be  little  affected  by  this,  as  it  does  not  depend  upon 
the  proliferation  of  the  products  of  syphilitic  inflammation. 
That  part  of  the  brain  in  which  necrosis  has  begun  will 
never  recover. 

In  occasional  instances  one  may  observe  the  complete 
recovery  of  an  arteritic  hemiplegia.  Fournier  states  that 
he  has  seen  ten  cases  of  arteritic  hemiplegia  which  recov- 
ered inside  of  a  few  weeks.  Hutchinson  describes  a  case 
of  arteritic  hemiplegia  in  which  the  recovery  still  remains 
good  after  ten  years. 

The  danger  in  syphilitic  arteritis  is  its  tendency  to 
relapse. 

The  memory  and  intelligence  are  not  as  apt  to  suffer  in 
specific  arteritis  as  in  arteriosclerosis  of  the  ordinary  type. 

The  prognosis  with  regard  to  life  is  also  better,  as  syph- 
ilitic arteritis  seldom  causes  death.  In  44  cases  which  came 
under  observation  no  fatality  was  caused  by  the  arteritis. 

The  paralysis  recovers  usually  more  fully  when  the 
patient  is  in  a  good  physical  condition  or  is  not  of  too 
advanced  an  age. 


PROGNOSIS  OF  SPECIFIC  BASILAR  MENINGITIS    161 

If  the  paralysis  after  from  two  to  four  weeks  shows  no 
indication  of  improvement,  one  may  conclude  as  in  the  non- 
specific hemiplegias  that  the  condition  will  be  permanent. 
An  arteritic  hemiplegia  will  recover  sooner  than  a  hemi- 
plegia,  a  triplegia,  or  a  pseudobulbar  paralysis,  because 
the  lesion  is  smaller.  The  appearance  of  contractures  indi- 
cates the  permanency  of  the  condition. 

In  my  own  44  cases  there  were  only  six  which  completely 
recovered  in  a  few  weeks;  in  the  others  a  greater  or  less 
degree  of  paresis  remained.  The  most  of  these  patients 
passed  out  from  under  my  observation.  In  16  cases  which 
I  was  able  to  follow  for  over  four  years,  five  relapsed,  three 
developed  other  forms  of  brain  syphilis,  two  general 
paresis,  and  six  have  remained  well,  except  for  a  slight 
residual  paralysis. 

The  prognosis  of  arteritic  disease  of  the  basilar  artery 
is  always  grave,  because  this  artery  supplies  the  most  im- 
portant vital  centres.  The  outlook  for  restitution  where 
the  arterial  affection  involves  the  nuclear  regions  is  also 
not  good,  as  has  been  shown  in  the  study  of  disease  of  the 
oculomotor  nucleus. 

Summary. — Owing  to  the  practical  importance  of  this 
subject  the  following  summary  in  tabular  form  has  been 
collected  from  my  hospital  and  private  practice  and  also 
from  the  literature. 

In  the  general  summary  table  by  the  term  "heredity" 
is  understood  the  occurrence  of  functional  neurosis  and 
psychoses  as  well  as  organic  disease  of  the  central  nervous 
system  in  the  antecedents;  by  the  "type  of  syphilis" 
whether  the  syphilis  has  appeared  in  severe  form  in  any  one 
of  its  three  stages  or  in  all  of  them,  and  whether  it  has 
been  refractory  to  treatment.  The  term  * '  interval ' '  indicates 
the  time  which  has  elapsed  between  the  initial  lesion  and 
the  appearance  of  the  nervous  affection.  By  "weakening 
factors"  are  meant  anaemia,  particularly  mental  and  physi- 
cal strains,  acute  and  chronic  psychic  and  physical  trauma, 
and  excesses  in  alcohol,  tobacco,  and  venery. 

The  185  cases  comprise  brain,  spinal,  and  cerebrospinal 
syphilis 
11 


162 


SYPHILIS  AND  THE  NERVOUS  SYSTEM 


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10-20  years 

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co 

PROGNOSIS  OF  SPECIFIC  BASILAR  MENINGITIS    163 


In  the  following  table  of  185  cases,  72  recovered,  30 
were  very  materially  improved,  and  122,  so  far  as  could 
be  determined,  suffered  no  relapse: 

TABLE  B 
Died  .................  16  cases  =  10.8  per  cent.  "I    AK  01  ., 

Unimproved  ..........  30  cases=20.3  per  cent.  }   '  >  •""-«"  P«  cent. 


Improved  .............  30  cases  =  20.3  per  cent.  \  -,  no 

Recovered  ............  72  cases=48.6  per  cent.  }  102 


per  cent. 


The  prognosis  is  presented  in  Table  C.  Under  the  term 
"good"  is  meant  recovered  or  very  much  improved,  and 
under  ''bad"  unimproved  or  very  little  improved  or  died. 

TABLE  C 


I.  Sex. 

Good. 

Bad. 

Male  

Per  cent. 

64  cases  =  68  .  1 

Per  cent. 
30  cases  —  31  9 

Female           

38  cases  =  70.4 

16  cases—  39  6 

II.  Age. 

Good. 

Bad. 

1—10  years         

Per  cent. 

2  cases  =  100 

Per  cent. 
0  case  —     00 

10-20  years             

0  case  =     0.0 

1  case  —100 

20-30  years  

13  cases  =  76  .  5 

4  cases  —  23  5 

30-40  years  

45  cases  =  77  .  6 

13  cases  —  22  4 

40—50  years         

29  cases  =  63.0 

17  cases  —  37  0 

50-60  years  

12  cases  =  63.2 

7  cases  —  36  8 

60-70  years  

0  case  =     0.0 

2  cases  —  100 

III.  Heredity. 

Good. 

Bad. 

Good  heredity  

Per  cent. 
71  cases  =  68.3 

Per  cent. 

33  cases—  31  7 

Poor  heredity      

13  cases  =  65.0 

7  cases  —  35  0 

IV.  Age  at  time  of  infection. 

Good. 

Bad. 

Under  40  years         

Per  cent. 

61  cases  =  70.1 

Per  cent. 

26  cases  —  29  9 

Over  40  years             

7  cases  =  63  .  7 

4  cases  —  36  4 

V.  Type  of  syphilis. 

Good. 

Bad. 

Severe  

Per  cent. 

15  cases  =  57  .  7 

Per  cent. 

1  1  cases  —  42  3 

Mild.              

15  cases  =  75  0 

5  cases  —  25  0 

Extragenital  

4  cases  =  57  1 

3  cases  —  42  9 

VI.  Treatment  of  syphilis. 

Good. 

Bad. 

Untreated  

Per  cent. 
32  cases  =  65  .  3 

Per  cent. 

17  cases  —  34  7 

Insufficient  treatment  

10  cases  =  71  .4 

4  cases  —  28  6 

Sufficient  treatment  

44  cases  =  68  .  7 

20  cases  —  31  3 

164 


SYPHILIS  AND  THE  NERVOUS  SYSTEM 


TABLE  C — Continued. 


VII.  Interval. 

Good. 

Bad. 

1-10  years  

Per  cent. 
45  cases  =  73.8 

Per  cent. 
16  cases  =  26  .  2 

Over  10  years.  . 

25  cases  =  64  .  1 

14  cases  =  35  .  9 

VIII.  Weakening  factors. 

Good. 

Bad. 

Lacking  

Per  cent. 

60  cases  =  73  .  2 

Per  cent. 

22  cases  =  26  .  8 

Present  

42  cases  =  63  .  6 

24  cases  =  36  .  4 

IX.  Form  of  disease  of  central 
nervous  system. 

Good. 

Bad. 

Cerebral  

Per  cent. 

67  cases  =  71.3 

Per  cent. 

27  cases  =  28  .  7 

Spinal  

16  cases  =  61  .  5 

10  cases  =  38  .  5 

Cerebrospinal  

19  cases  =  67  .  9 

9  cases  =  32  .  1 

Arterial  

20  cases  =  66  .  6 

10  cases  =  33  .  9 

Meningeal  

72  cases  =  69  .  2 

32  cases  =  30  .  8 

Meningeal  and  arterial.  .  .  . 

10  cases  =  71.4 

4  cases  =  28.6 

X.  Relapses. 

Good. 

Bad. 

No  relapses  

Per  cent. 

71  cases  =  76.3 

Per  cent. 
22  cases  =  23  .  7 

One  relapse  

17  cases  =  58  6 

12  cases  =  41  .4 

Several  relapses  

14  cases  =  60  .  9 

9  cases  =  39  .  1 

In  regard  to  the  individual  factors  which  influence  the 
prognosis,  I  wish  to  make  the  following  observations : 

First.     Sex  has  no  influence  on  the  prognosis. 

Second.  The  prognosis  is  best  between  the  ages  of 
twenty  and  forty.  After  forty  it  becomes  at  first  slowly 
poorer  and  then  later  rapidly  so.  In  old  age  the  outlook  is 
entirely  bad. 

Third.  According  to  my  experience,  the  prognosis  in 
the  cases  with  a  bad  nervous  inheritance  is  not  as  good  as 
in  the  cases  without  it. 

Fourth.  The  prognosis  becomes  worse  in  cases  where 
the  syphilis  was  contracted  after  the  age  of  forty. 

Fifth.  In  my  own  cases  where  the  disease  had  shown 
a  severe  form  in  the  primary  and  secondary  stages  there 
was  also  a  tendency  to  a  more  severe  course  in  the  nervous 
system  than  in  those  cases  where  the  early  syphilis  had 
been  of  a  mild  type. 


Sixth.  Although  astonishing,  it  seems  to  be  a  pretty 
definitely  settled  fact  that  the  prognosis  in  those  cases 
which  have  either  received  no  treatment  or  insufficient 
treatment  appears  just  as  good  as  in  those  cases  which 
have  received  thorough  treatment. 

Seventh.  When  the  interval  between  the  time  of  the 
infection  and  the  outbreak  of  the  nervous  symptoms  is  over 
ten  years  the  influence  on  the  prognosis  is  unfavorable. 

Eighth.  The  weakening  factors  exert  an  unfavorable 
influence  on  the  prognosis. 

Ninth.  There  appears  to  be  no  essential  difference  in 
the  prognosis  between  syphilis  of  the  brain,  spinal  cord,  and 
cerebrospinal  lues. 

Tenth.  The  prognosis  is  better  in  cases  which  show  no 
relapses.  A  number  of  relapses  do  not  seem  to  decrease 
the  chances  of  the  final  outcome  in  comparison  with  the 
cases  which  have  suffered  one  relapse. 

Table  D  gives  the  year  of  the  relapse  dating  from  the 
time  of  the  nervous  outbreak. 

TABLE  D 

First  year 20  cases  =31.7  per  cent. 

Second  year 21  cases  =  33.3  per  cent. 

Third  year 9  cases  =  14.3  per  cent. 

Fourth  year 5  cases  =  7.9  per  cent. 

Seventh  year 1  case  =   1.6  per  cent. 

Tenth  year 2  cases  =  3.2  per  cent. 

Sixteenth  year 1  case  =   1.6  per  cent. 

Twenty-second  year 2  cases  =  2.3  per  cent. 


VIII 

NEUROSES  AND  PSYCHOSES  IN  SYPHILITICS 
AND  IN  CEREBRAL  SYPHILIS 

IN  the  consideration  of  brain  lues,  psychic  disturbances 
for  several  reasons  merit  special  attention:  First,  because 
in  true  syphilis  the  disturbances  of  the  intellectual  activity 
are  frequent  and  manifold;  second,  because  without  gross 
pathological  lesions  in  the  brain  in  syphilitics  various  men- 
tal affections  of  typical  and  atypical  forms  may  occur; 
and,  third,  because  the  exceedingly  important  ailment,  de- 
mentia paralytica,  in  the  great  majority  of  the  cases  is  to 
be  regarded  as  a  postsyphilitic  disease. 

Historical. — It  has  been  recognized  for  a  long  time  that 
the  psychic  functions  may  be  disturbed  in  cerebral  lues. 
Bell  has  reported  a  case  of  chronic  mania  in  a  syphilitic 
woman  who  suffered  with  headache  and  epilepsy  and  was 
relieved  of  both  affections  by  the  administration  of  mercury. 

Cases  of  cerebral  syphilis  do  occur  in  which  no  real 
psychic  disturbances  whatever  are  manifested;  usually, 
however,  close  observation  will  reveal  mild  psychic  anoma- 
lies in  the  majority  of  the  cases. 

It  has  also  been  recognized  for  a  long  time  that  the 
so-called  " functional  psychoses"  (cases  of  brain  syphilis 
are  not  included  in  this  category)  can  occur  in  luetics. 
Lagneau  was  the  first  to  direct  our  attention  to  this.  He 
divided  the  functional  psychoses  in  syphilitics  into  melan- 
cholia, mania,  dementia,  and  idiocy. 

The  question  of  chief  importance  in  regard  to  this  sub- 
ject is  to  determine  whether  the  syphilitic  virus  in  itself 
can  so  alter  the  brain  that  it  causes  the  development  of  a 
psychosis  which  may  be  said  to  be  characteristic  of  syph- 
ilis, and  may  be  distinguished  as  such  from  the  psychoses  of 
non-syphilitics. 

The  " simple  or  functional  psychosis"  first  mentioned 
by  Lagneau  was  also  as  such,  occurring  in  syphilis,  recog- 

166 


NEUROSES  AND  PSYCHOSES  167 

nized  later  by  a  large  number  of  observers,  among  whom 
were  Albers,  Erlenmeyer,  and  Jolly.  Erlenmeyer  has  writ- 
ten an  especially  comprehensive  monograph  concerning 
syphilis  and  mental  diseases,  in  which  he  divides  the  mental 
affections  in  lues  into  the  so-called  simple  or  functional 
psychoses,  the  psychoses  which  are  complicated  with  dis- 
turbances of  motion  and  sensation,  and  the  various  types  of 
general  paresis. 

Fournier  distinguished  two  conditions,  a  chronic  depres- 
sive state  with  a  gradually  developing  loss  of  the  intellect, 
and  acute  type  with  a  greater  or  less  degree  of  excitement 
in  the  form  of  delirium  and  mania,  which  latter  he  attrib- 
uted to  brain  irritation. 

Heubner  recognized  likewise  the  occurrence  of  a  simple 
or  functional  psychosis  in  syphilitics  and  in  addition  dif- 
ferentiated the  disturbances  of  the  psychic  functions  into 
the  three  forms  of  organic  specific  brain  disease  as  classified 
by  him. 

In  gummatous  disease  of  the  brain  convexity,  according 
to  Heubner,  there  predominates  a  condition  of  depression 
and  irritability  which  may  develop  into  either  a  deep  melan- 
cholic or  maniacal  state,  and  is  not  infrequently  followed  by 
loss  of  memory  and  intelligence,  as  well  as  changes  in  the 
disposition. 

Corresponding  to  the  location  of  the  pathological  lesion 
this  condition  is  combined  with  the  various  forms  of  aphasia 
and  cortical  irritations  and  paralyses.  In  the  arteritis  of 
the  basilar  arteries  Heubner  states  that  one  is  more  apt 
to  encounter  mental  feebleness,  while  in  arteritis  on  the 
convexity  the  mental  symptoms  exhibited  are  similar  to  an 
intoxicated  condition,  a  condition  of  semiconsciousness 
where  the  patient  undertakes  and  carries  out  certain  acts 
while  in  this  dazed  state. 

This  form  of  impairment  of  the  intellectual  capacity 
is  characterized  also  by  the  accompanying  symptoms  of 
paralysis  and  cortical  irritation. 

The  relationship  which  exists  between  lues  and  the  so- 
called  functional  neuroses,  neurasthenia  and  hysteria,  has 
also  received  much  attention. 


168  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

The  Different  Ways  in  Which  Syphilis  Causes  Psychic  Dis- 
turbances.— There  are  various  ways  in  which  a  syphilitic 
infection  can  produce  disturbances  of  the  mental  activity. 
If  we  regard  the  psychic  impairment  as  an  expression  of 
the  disturbances  of  nutrition,  then  these  disturbances  may 
be  brought  about  in  one  of  two  ways :  either  by  a  change  in 
the  blood  itself,  or  by  disease  of  the  passages  through 
which  the  blood  is  conducted  to  the  tissues. 

The  nervous  elements  may  also  be  damaged  by  the  post- 
syphilitic  poison. 

Psychic  causes  which  are  connected  with  the  specific 
infection  may  produce  mental  disturbances  in  a  brain  whose 
blood  and  circulatory  paths  are  already  impaired. 

Furthermore,  one  should  consider  whether  long-con- 
tinued mercurial  treatment  may  be  regarded  as  having  a 
deleterious  effect  upon  the  brain  by  causing  nutritional 
disturbances. 

Changes  in  the  Blood. — Virchow  was  one  of  the  first  to 
direct  attention  to  the  changes  which  occur  in  the  blood 
as  the  result  of  syphilis.  He  demonstrated  a  diminution  in 
the  number  of  blood-corpuscles  and  an  increase  in  the  albu- 
minous constituents.  Martin  and  Hiller  found  that  the 
number  of  red  blood-cells  was  greatly  reduced  in  the  sec- 
ondary stage,  and  that  also  independent  of  this  there  was  a 
diminution  in  the  amount  of  haemoglobin.  On  the  other 
hand,  Anz  found  in  addition  to  the  decrease  in  the  number 
of  red  cells  an  increase  in  the  white  corpuscles  in  the  sec- 
ondary stage,  so  that  one  can  speak  of  a  relative  and 
absolute  specific  leucocytosis.  Other  changes  in  the  white 
cells  have  also  been  determined,  such  as  a  decrease  in  the 
polynuclear  leucocytes  and  an  increase  in  the  mononuclears 
and  transitional  cells. 

An  increase  in  the  eosinophiles  has  likewise  been  noted. 

All  these  changes  are  characteristic,  in  that  they  are 
produced  by  syphilis,  that  they  appear  only  after  the  spe- 
cific infection,  and  that  they  vary  in  intensity  with  the 
reappearance  or  disappearance  of  new  secondary  symp- 
toms, and  can  be  influenced  and  a  normal  condition  restored 
by  antispecific  treatment. 


NEUROSES  AND  PSYCHOSES  169 

The  French  were  among  the  first  to  lay  emphasis  upon 
a  syphilitic  chlorosis  which  was  not  benefited  by  the  custo- 
mary administration  of  iron  and  other  tonics,  but  yielded 
only  to  mercurial  treatment. 

This  blood  condition  may  manifest  itself  before  the  out- 
break of  the  first  eruption,  or  during  the  second  incubation 
period,  and  Stille  has  found  it  even  at  the  time  of  the 
primary  lesion. 

Dyscrasia  as  a  Result  of  Antispecific  Therapy. — It  must  be 
acknowledged  that  the  blood  dyscrasia  sometimes  is  pro- 
duced by  too  frequently  repeated  and  too  prolonged  courses 
of  mercury  and  iodid.  In  such  cases  we  have  an  artificially 
created  toxic  dyscrasia.  Patients  behave  very  differently 
in  regard  to  mercury  and  potassium  iodid.  Some  can  take 
large  doses  of  both  for  a  long  time  and  show  improvement 
in  their  blood  and  nutrition,  others  are  able  to  tolerate 
these  drugs  only  for  a  certain  time,  and  still  others  manifest 
an  idiosyncrasy  from  the  very  beginning  against  antispe- 
cific  therapy.  For  this  reason  it  not  infrequently  happens 
that  it  becomes  necessary  for  one  to  pause  and  consider 
whether  the  existing  dyscrasia  and  state  of  poor  nu- 
trition are  caused  by  the  syphilis  or  by  the  antispecific 
treatment. 

Disease  of  the  Blood  Channels. — The  blood  channels,  as  has 
been  stated  before,  may  likewise  suffer  in  brain  syphilis. 
Secondarily  also  the  intra-  and  extravascular  lymph  spaces, 
and  by  chronic  inflammatory  disease  of  the  leptomeninges 
and  the  consequent  influence  of  the  relationship  between 
the  intracerebral  lymph  channels  with  the  epicerebral  space, 
a  disturbance  of  the  permeability  of  the  lymph  channels  in 
the  entire  brain  may  result.  In  this  way  the  exchange  be- 
tween the  lymph  and  the  blood  is  retarded,  and  sometimes 
prevented,  so  that  by  the  retardation  of  both  the  removal 
and  replacement  of  the  waste  products  of  metabolism  a 
disturbance  of  the  nutrition  of  the  nervous  elements  takes 
place. 

The  Influence  of  the  Infection  Itself  upon  the  Mental  Con- 
dition.— The  specific  infection  may  often  be  regarded  as  a 
psychic  trauma,  as  in  the  etiology  of  every  mental  affection, 


170  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

sorrow,  care,  anxiety,  and  fright  play  an  important  role ;  so 
the  depressing  influences  attending  an  infection  of  syphilis 
should  be  considered  as  important  in  the  causation  of  the 
mental  disturbances  following,  in  so  far  as  they  are  not 
due  to  organic  changes. 

Native  Resistance  of  the  Person  Infected. — Binswanger,  as 
well  as  other  authors,  has  repeatedly  stated  that  it  depends 
very  much  upon  the  vitality  of  the  patient  as  to  whether  cer- 
tain injuries  of  the  nervous  system  will  produce  a  func- 
tional or  organic  psychosis.  Accordingly  as  the  individual 
resistance  of  the  nerve-cell  itself,  through  impairment  of 
nutrition  or  psychic  trauma,  is  affected;  if  only  the  Nissl 
bodies,  which  are  thought  at  the  present  time  to  represent 
the  nourishing  substance  of  the  cell,  and  which  after  great 
destruction  may  be  again  replaced,  are  affected,  or  if  the 
function-producing  elements  of  the  cells  are  damaged  in 
an  irreparable  way.  In  the  first  instance  we  would  have 
the  picture  of  a  simple  exhaustion  which  is  capable  of 
reparation,  in  the  second  that  of  the  progressive  organic 
psychosis. 

Functional  Nervousness. — One  observes  not  infrequently 
that  hitherto  healthy  persons,  after  a  luetic  infection,  ex- 
hibit symptoms  of  general  nervousness.  They  lose  their 
steadiness  in  thinking  and  accomplishing,  are  irritable,  their 
sleep  is  disturbed.  They  complain  often  of  rheumatoid  in- 
definite pains,  which  are  localized  sometimes  in  one  place 
and  sometimes  in  another.  Accompanying  these  symptoms 
is  a  feeling  of  pressure  on  the  head.  We  may  regard  such 
a  condition  either  as  the  consequence  of  the  above-described 
blood  alteration,  or  as  the  expression  of  the  slowly-develop- 
ing changes  in  the  arteries  and  the  resulting  diminution  in 
the  blood  supply  to  the  nerve  substance,  or  as  the  precur- 
sory symptoms  of  a  paresis.  Fournier  and  Lang  have 
stated  that  this  vague  nervousness  in  luetic  patients  is  to 
be  regarded  as  the  most  frequent  clinical  expression  of  a 
meningeal  irritation.  However  that  may  be,  the  fact  re- 
mains that  such  conditions  often,  under  antispecific  therapy, 
disappear  without  the  appearance  of  any  other  symptoms 
of  syphilis. 


NEUROSES  AND  PSYCHOSES  171 

Cerebral  Neurasthenia. — Cerebral  neurasthenia  comes 
next  for  our  consideration.  The  chief  etiological  factors 
standing  in  causal  relationship  to  cerebral  neurasthenia 
or  psychasthenia  in  luetics  are  the  shock  of  the  infection 
itself,  combined  with  the  general  disturbances  of  nutrition, 
alcoholic  excesses,  an  inherited  lessened  resistance  of  the 
nerve-cell,  and  prolonged  mercurial  therapy. 

The  affection  in  syphilitics  does  not  differ  from  a 
psychasthenia  in  non- syphilitics.  Antispecific  treatment  in 
some  instances — not  in  all,  however — combined  with  gen- 
eral constructive  measures,  has  seemed  to  me  to  produce  a 
quicker  effect  than  we  are  accustomed  to  see  otherwise  in 
this  class  of  cases  in  non-luetics. 

The  following  case  of  cerebral  neurasthenia  corrobo- 
rates this  fact: 

A  gentleman,  forty-five  years  old,  who  ten  years  before 
had  contracted  syphilis,  had  complained  for  a  long  time 
of  pressure  on  the  head, difficulty  in  concentrating  his  mind 
on  his  work,  feelings  of  apprehension,  and  a  sense  of  oppres- 
sion in  the  breast.  He  was  also  easily  exhausted  by  every 
attempt  at  physical  and  mental  exertion.  He  had  taken  a 
number  of  courses  of  treatment  in  hydrotherapy  and  diet, 
also  psychotherapy,  without  result.  A  six  weeks'  course 
of  inunctions  with  potassium  iodid  brought  about  a  won- 
derful improvement  without  any  other  treatment  and  with- 
out any  interference  with  his  occupation. 

Neurasthenia  in  luetics  is  most  apt  to  appear  during  the 
secondary  stage.  In  the  differential  diagnosis  between  sim- 
ple nervousness  and  neurasthenia  in  patients  who  have  had 
syphilis  and  beginning  paresis,  the  four  reactions  must  be 
regarded  as  of  the  utmost  importance. 

Hysteria. — Hysteria  is  also  not  infrequently  observed  in 
syphilitics.  Charcot  regards  lues  with  reference  to  hysteria 
as  analogous  to  other  poisons,  especially  to  the  lead-alcohol- 
arsenic  intoxications.  Likewise,  as  in  other  organic  brain 
and  spinal-cord  lesions,  so  in  specific  disease  of  the  nervous 
system  hysteria  may  be  an  accompanying  symptom. 

One  occasionally  sees  patients  who  have  become  cachec- 
tic through  a  prolonged  mercurial  treatment  develop  hys- 


172  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

terical  symptoms.  In  order  to  attribute  the  hysteria  to 
syphilis  it  is  necessary  that  the  patients  before  their  infec- 
tion have  never  exhibited  any  symptoms  of  hysteria,  also 
that  the  hysteria  develops  during  the  time  of  the  syphilis 
and  can  be  either  improved  or  cured  by  antispecific  treat- 
ment. 

In  the  diagnosis  of  hysteria,  however,  great  caution  is 
necessary,  to  which  Wunderlich,  Wagner,  and  Heubner 
have  repeatedly  called  attention. 

The  symptoms  produced  by  the  specific  organic  changes 
in  the  brain  are  often  so  transient  in  character,  and  come 
and  go  so  quickly,  that  the  mistake  is  frequently  made  of 
diagnosing  them  as  hysterical. 

The  following  case  illustrates  the  relationship  existing 
between  syphilis  and  hysteria: 

A  lady,  thirty-eight  years  old,  who  had  never  manifested 
the  slightest  symptoms  of  nervousness  or  hysteria,  six 
months  after  a  syphilitic  infection  developed  a  typical  hys- 
teria major  which  proved  refractory  to  the  rest  cure  and 
hydrotherapy,  but  responded  splendidly  to  mercurial  treat- 
ment. The  hysteria  has  never  returned  to  the  patient, 
although  she  has  since  undergone  a  great  deal  of  mental 
strain  and  sorrow. 

All  observers  agree  that  in  so  far  as  the  clinical  picture 
is  concerned  hysteria  in  syphilis  does  not  differ  in  any  way 
from  the  ordinary  forms  of  the  disease. 

Chorea. — Concerning  the  relationship  between  chorea  and 
syphilis,  only  a  few  cases  have  been  reported.  Zambaco 
has  described  a  case  in  which  the  chorea  developed  in  a 
previously  healthy  person  without  any  other  assignable 
cause,  and  yielded  promptly  to  anti syphilitic  treatment 
after  other  non-specific  therapy  had  been  tried  without  re- 
sult. Kowalewsky  has  reported  a  family  in  which  all  the 
children  of  a  syphilitic  father  suffered  with  chorea.  May- 
erhofer,  Salinger,  and,  recently,  Gennanus  Flatau  also 
have  reported  cases  which  one  must  consider  as  syphilitic 
chorea. 

Epilepsy. — Epilepsy,  on  the  contrary,  stands  in  close 
relationship  to  a  specific  infection.  In  speaking  of  epilepsy 


NEUROSES  AND  PSYCHOSES  173 

here,  those  cases  of  symptomatic  epilepsy  which  have  been 
referred  to  before,  as  well  as  the  cases  of  partial  and  gen- 
eral epileptic  convulsions,  which  are  the  precursors  of 
later-developing  severe  organic  brain  syphilis,  and  the  epi- 
leptic attacks  which  sometimes  occur  as  the  first  serious 
expression  of  a  general  paresis,  are  excluded. 

This  is  the  kind  of  epilepsy  which  cannot  be  distin- 
guished from  the  idiopathic  form.  Fournier  especially  has 
directed  our  attention  to  this  type,  and  every  one  who  has 
seen  many  cases  of  epilepsy  knows  that  frequently  a  syph- 
ilitic infection  stands  alone  as  the  only  etiological  factor. 

This  parasyphilitic  epilepsy  occurs  often  in  persons 
where  the  specific  infection  took  place  many  years  ago,  but 
also,  sometimes,  is  found  in  the  earlier  stages  of  the  dis- 
ease. The  attacks  appear,  in  general,  less  often  than  in  the 
ordinary  epilepsy,  the  intelligence  is  also  apt  to  be  less 
affected  than  in  idiopathic  epilepsy,  and  in  a  few  cases  the 
response  to  active  specific  treatment  is  good. 

Slight  attacks  of  petit-mal  sometimes  occur  in  between 
the  severe  attacks.  These  postsyphilitic  epileptic  neuroses 
may  be  differentiated  from  the  specific  organic  forms,  in 
that  they  do  not  resemble  the  character  of  a  cortical  epi- 
lepsy, are  not  accompanied  by  other  cerebral  phenomena, 
may  persist  for  a  long  time  without  changing  their  charac- 
ter, and  also  by  the  fact  that  the  effect  of  antispecific  treat- 
ment on  them  is  uncertain. 

Vogt  and  Binswanger  regard  this  form  of  epilepsy  as 
due  to  the  strengthening  of  a  hereditary  epileptic  tendency 
by  lues,  and  the  awakening  of  a  latent  epileptic  predisposi- 
tion through  acquired  syphilis.  Both  of  these  authorities 
consider  this  form  of  epilepsy  to  be  of  rare  occurrence. 
In  my  own  experience  I  have  had  the  opportunity  of  observ- 
ing up  to  1902  twelve  cases.  In  all  of  these  the  history  of 
infection  was  well  established,  there  was  no  inherited  tend- 
ency, no  head  injury,  and  no  history  of  the  use  of  alcohol. 
The  attacks  appeared  quite  regularly  at  intervals  varying 
from  two  to  six  months.  There  were  no  intercurrent 
psychic  anomalies  in  any  of  the  cases.  In  all  of  them  re- 
peated treatment  with  mercury,  and  especially  potassium 


174  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

iodid,  was  without  influence.  In  one  case  the  specific  infec- 
tion dated  back  six  months,  in  two,  one  year,  and  in  one  case 
each,  five,  seven,  and  twelve  years  respectively. 

Such  types  of  epilepsy  are  to  be  considered  in  the  same 
category  with  tabes  and  paresis  as  post-  or  parasyphilitic 
disease,  the  causative  factor  of  which  is  a  nerve  poison  pro- 
duced secondarily  as  the  result  of  syphilis. 

Hypochondria. — One  also  finds  in  syphilitics  hypochon- 
dria. In  its  simplest  forms  it  is  a  well-expressed  syphilo- 
phobia.  The  patients  perceive  in  every  sensation  of  discom- 
fort, of  transient  headache,  of  forgetfulness  of  a  word  or 
promise  or  conversation  the  beginning  symptoms  of  brain 
softening.  They  think  they  have  discovered  symptoms  of 
syphilis  on  their  bodies,  they  observe  with  a  morbid  con- 
scientiousness all  of  their  physical  functions,  and  become, 
through  increasing  complaints  concerning  their  mental  and 
physical  condition,  a  nuisance  to  those  with  whom  they  come 
in  contact. 

Such  cases  are  not  infrequent  and  occur  almost  always 
among  the  educated  classes.  This  is  a  field  for  the  highest 
skill  of  the  physician.  On  the  one  hand,  the  thoughts  of 
the  patients  must  be  diverted  from  their  disease  and  its 
consequences;  on  the  other,  the  ensemble  of  the  case  may 
require  energetic  and  repeated  antispecific  therapy. 

In  severe  cases  one  finds  hypochondriacal  paranoic  in- 
terpretations of  organic  sensations  and  well-expressed 
hypochondriacal  compulsory  ideas,  which  I  have  observed 
in  three  cases  to  degenerate  into  severe  paranoia  hypo- 
chondriaca. 

These  conditions  present  nothing  which  in  themselves 
are  characteristic,  because  they  are  also  found  in  persons 
who  have  never  had  syphilis.  One  of  these  cases  of  syph- 
ilophobia  came  under  my  care  some  time  ago.  All  efforts 
made  to  build  up  the  nervous  system  through  tonics,  hydro- 
therapy,  and  psychotherapy  failed,  while  a  course  of  anti- 
specific  treatment  brought  about  a  satisfactory  result. 

The  frequency  of  hypochondriacal  states  and  hyTpo- 
chondriacal  mental  disturbances  in  syphilitics  has  long  been 
known. 


NEUROSES  AND  PSYCHOSES  175 

Melancholia. — This  kypochondriacal  mood  sometimes  de- 
velops into  a  true  melancholia.  All  observers  agree  that 
of  the  pure  disturbances  of  the  emotions  in  syphilis  melan- 
cholia is  the  most  frequent.  The  melancholia  may  manifest 
itself  at  first  as  a  simple  depression  and  then  pass  through 
all  the  grades  to  the  most  severe  forms  with  compulsory 
suicidal  impulses. 

In  the  clinical  picture  presented  there  is  nothing  which 
is  characteristic  of  syphilis.  There  is  no  such  an  entity  as 
a  syphilitic  melancholia.  We  only  know  that  it  may  de- 
velop in  any  of  the  stages  of  syphilis  and  also  long  after 
all  tangible  forms  of  the  disease  have  passed  away.  How- 
ever, one  quite  often  sees  the  melancholia  in  syphilis  dis- 
appear under  antispeciiic  therapy. 

Mania. — Mania  also  occurs  in  syphilitics  who  otherwise 
do  not  show  any  evidences  of  the  disease,  and  who  have 
a  good  nervous  inheritance  and  are  without  a  previous  his- 
tory of  maniacal  attacks. 

Attacks  of  maniacal  excitement,  however,  in  organic 
specific  brain  affections  are  much  more  often  observed, 
whether  the  disease  is  one  of  the  convexity,  base,  or  a  gen- 
eral syphilitic  arteritic  disease. 

I  have  observed  typical  maniacal  attacks  in  two  cases 
with  symptoms  of  a  basilar  gummatous  meningitis.  The 
mania  manifested  itself  in  an  intense  restlessness,  a  typical 
flood  of  ideas  without  evidence  of  mental  feebleness,  an 
exaggerated  self-consciousness,  and  strong  animal  desires. 

The  following  case  is  one  of  severe  mania,  ending  in 
death : 

The  specific  infection  dated  back  two  years.  The 
patient  was  a  sober  and  industrious  young  man.  Without 
any  apparent  cause  he  developed  a  condition  of  exhilaration 
which  rest  in  bed  did  not  relieve.  The  exalted  state  soon 
passed  into  one  of  a  typical  mania,  which  made  it  necessary 
to  transfer  him  to  the  hospital.  Here  the  maniacal  con- 
dition increased  in  intensity  and  was  accompanied  by  an 
increasing  stream  of  ideas  on  the  grandiose  order.  After 
four  days  in  the  hospital  the  patient  became  disoriented  and 
confused.  He  refused  nourishment  and  had  to  be  tube-fed. 


176  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

He  grew  rapidly  worse  and  died  ten  days  from  the  begin- 
ning of  his  sickness. 

At  the  autopsy  the  internal  organs  were  found  to  be 
normal.  Macro  scopically  the  brain  also  appeared  normal, 
except  for  a  general  hypera3mia.  The  microscopic  examina- 
tion showed  the  capillaries  to  be  overdistended  with  blood, 
with  extensive  perivascular  cell  infiltration  and  cellular 
clumps  in  the  nerve  substance.  No  anomalies  of  the  gan- 
glion cells  or  of  the  glia  were  discovered. 

In  maniacal  attacks  in  syphilitics  one  should  always 
keep  in  mind  the  possibility  that  they  may  be  the  first 
warnings  of  a  beginning  paresis.  Usually,  however,  by  the 
observation  of  organic  symptoms,  and  especially  by  the 
demonstration  of  mental  and  moral  delinquencies,  one  is 
able  to  detect  in  the  patient  the  paresis  which  had  remained 
latent  up  to  the  time  of  the  maniacal  outbreak.  Such  cases 
are  inclined  to  run  a  very  acute  course,  even  what  might 
be  called  "galloping"  in  character. 

It  may  be  pertinent  to  state  here  that  the  four  reactions, 
even  in  the  initial  stages  of  paresis,  are  positive. 

Eecently  A.  Westphal  has  called  attention  to  a  psychic 
condition  which  is  manifested  by  a  permanent  exalted  hypo- 
maniacal  state,  with  exaggerated  self-consciousness  and 
various  and  changeable  grandiose  ideas.  In  spite  of  the 
loss  of  the  pupil  reflexes  to  light  and  increase  of  the  tendon 
reflexes,  Westphal  assumes  that  these  cases  do  not  belong 
in  the  category  of  paresis  while  the  change  in  personality 
is  not  marked  enough  and  while  no  progressive  dementia 
develops;  also  the  powers  of  observation  and  speech  re- 
main intact.  Westphal  believes  that  minute  changes  in  the 
brain,  perhaps  depending  upon  specific  arterial  disease,  or 
upon  the  effect  of  a  toxin  causing  nutritional  disturbances 
in  the  nerve  tissue,  are  responsible  for  these  conditions. 

Manic-depressive  Insanity. — The  circulatory  type  of  psy- 
chosis, manic-depressive  insanity,  occurs  likewise  in  luetics. 

I  wish  to  call  attention  here  to  two  cases  of  this  form  of 
mental  trouble,  both  of  which  presented  pupil  anomalies  and 
symptoms  of  degeneration  of  the  posterior  column.  These 


NEUROSES  AND  PSYCHOSES  177 

objective  symptoms  in  these  cases  did  not  indicate,  as  their 
subsequent  history  showed,  general  paresis,  but  simply 
after-effects  of  a  former  specific  infection. 

The  first  case  was  in  a  high  official,  fifty  years  old,  who 
before  his  marriage  had  syphilis.  He  was  especially  gifted 
mentally  and  in  his  particular  work  had  acquired  consider- 
able reputation.  Six  years  ago  he  became  hypornaniacal, 
then  was  quarrelsome  and  hard  to  get  along  with,  then  en- 
gaged in  spasmodic  schemes  and  combinations,  then  became 
lavish  in  spending  money.  As  a  result  of  contention  with 
his  superiors  he  lost  his  position.  He  had  Argyll-Eobertson 
pupils  and  loss  of  both  knee  reflexes,  as  well  as  zones  of 
hypoalgesia  on  the  legs  and  feet. 

My  impression  at  this  time  was  that  he  had  paresis. 
In  a  few  days  his  mental  state  changed  entirely  to  one  of 
depression.  This  continued  for  about  six  months,  when  an 
exalted  condition  again  appeared.  At  the  same  time  there 
existed  true  changes  of  character.  The  once  sensitive,  tact- 
ful man  became  brutal  and  reckless.  He  drank  and  cohab- 
ited to  excess  and  bragged  over  his  liaisons.  We  still 
thought  the  case  one  of  paresis. 

The  case  continued  as  one  of  an  alternating  manic- 
depressive  psychosis.  The  patient  lived  eight  years  and 
was  continually  in  either  a  maniacal  or  depressive  state. 
Disturbances  of  intelligence,  speech,  or  exalted  delusive 
ideas  never  were  manifested.  The  patient  died  with  an 
intercurrent  pneumonia.  At  the  time  of  his  death  my  diag- 
nosis was  that  of  manic-depressive  insanity  in  a  luetic,  with 
abortive  tabes. 

The  microscopical  examination  of  the  spinal  cord  re- 
vealed quite  a  well-advanced  tabes.  In  the  brain  there  were 
no  paretic  changes.  The  microscopical  examination  of  the 
cortex  in  different  areas  showed  simple  changes  in  the  walls 
of  the  arteries  and  in  places  increase  of  the  glia. 

Another  case  was  in  a  man  fifty-three  years  old.    His 

specific  infection  dated  back  twenty  years.    He  began  six 

years  ago  to  have  maniacal  attacks.     The  diagnosis  of  a 

typical  paresis  was  made  in  a  university  clinic.    This  dis- 

12 


178  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

couraged  him  and  plunged  him  into  a  depression  which 
lasted  a  number  of  months  and  was  followed  by  a  severe 
exalted  state. 

I  saw  the  patient  in  this  exalted  state,  which  bore  a 
maniacal  character.  Objectively  he  had  anisocoria  and 
loss  of  the  pupil  reactions  to  light,  along  with  active  tendon 
reflexes.  There  was  no  disturbance  of  speech  or  symptoms 
of  dementia.  Since  my  first  examination  the  patient  has 
twice  changed  from  depression  to  mania  and  mania  to 
depression.  No  new  organic  symptoms  have  appeared,  and 
there  is  still  no  decrease  in  the  intelligence. 

These  two  observations  serve  to  prove  that  Thomsen's 
assertion  that  a  case  of  manic-depressive  insanity  may  re- 
semble a  paresis,  except  that  the  anamnesis  and  the  pres- 
ence or  absence  of  physical  symptoms  determines  the  diag- 
nosis, is  not  absolutely  true. 

Paranoia. — Paranoia  has  been  observed  in  syphilitics, 
both  as.  a  primary  psychosis  and  with  the  accompanying 
symptoms  of  specific  organic  brain  disease.  A  genuine 
paranoia  is,  however,  as  Jolly  has  pointed  out,  a  very  rare 
specific  psychosis.  Jolly  states  that  in  the  later  stages  of 
the  dementia  type  of  syphilis  there  is  a,  tendency  to  certain 
delusions,  but  that  these  are  not  fixed  and  not  systematized. 

A  merchant,  twenty-four  years  old,  who  one  and  a  half 
years  previously  had  contracted  syphilis,  came  under  my 
care  because  of  a  severe  specific  paralysis  of  the  cranial 
nerves.  After  a  short  period  of  excitement  he  exhibited  for 
a  period  of  two  months  systematized  delusions  of  persecu- 
tion. He  thought  that  his  bride  had  been  infected  with 
syphilis  by  the  physician  who  was  attending  him,  and  for 
that  reason  the  physician  was  trying  to  poison  him  by  put- 
ting poison  in  his  food  and  thus  getting  him  out  of  the  way. 
Under  antispecific  therapy  the  nerve  paralysis  and  delus- 
ions gradually  disappeared. 

Catatonia. — I  had  never  seen  the  condition  of  catatonia 
either  in  brain  lues  or  in  luetics  until  recently.  A  few 
months  ago  I  saw  this  psychosis  in  a  patient  whose  parents 
had  before  the  conception  of  the  patient  acquired  syphilis. 


NEUROSES  AND  PSYCHOSES  179 

A.  Westphal  has,  however,  reported  one  case  of  catatonia 
which  occurred  during  the  course  of  a  cerebral  syphilis. 

Amentia. — Amentia  has  been  observed  in  syphilis.  Jolly 
describes  amentia  as  the  chief  representative  of  the  intoxi- 
cation psychoses.  The  entire  inability  of  the  patients  to 
receive  new  impressions,  the  acute  and  complete  loss  of 
memory,  the  acute  confusion  with  delirium,  and  the  succes- 
sion of  hallucinations,  are  the  important  clinical  features. 
As  syphilis  is  a  constitutional  poison,  it  is  to  be  expected 
that  this  form  of  mental  disturbance  would  occur.. 

It  has  been  observed  with  and  without  pathological 
changes.  It  is  often  difficult  to  determine,  because  of  the 
so-frequent  occurrence,  in  the  same  patient,  of  chronic 
alcoholism  and  syphilis,  how  much  to  attribute  to  the  one 
factor  and  how  much  to  the  other.  The  somatic  symptoms 
of  alcoholism,  the  general  habitus,  the  characteristic  motor 
unrest,  changes  in  the  internal  organs  which  accompany 
chronic  alcoholic  intoxication,  and,  above  all,  the  more  acute 
and  rapid  course,  with  the  more  frequent  tendency  to  recov- 
ery, are  to  be  regarded  as  the  deciding  factors.  It  is  im- 
portant to  remember  that  Korsakoff's  psychosis,  a  psy- 
chosis often  accompanied-by  a  polyneuritis  originating  from 
an  alcoholic  basis,  represents  an  acute  amentia.  This  psy- 
chosis also  frequently  occurs  in  alcoholics.  In  nervous  dis- 
ease due  to  alcoholism,  however,  although  the  patient  has 
had  lues,  the  three  reactions  in  the  spinal  fluid  are  negative. 

Dementia. — Much  more  common  than  the  amentia,  and 
the  most  frequent  form  of  mental  disturbance  in  syphilis, 
is  the  dementia.  Dementia  as  a  result  of  syphilis  may  arise 
from  different  causes.  It  may  develop  as  the  termination  of 
the  functional  psychoses,  melancholia,  mania,  and  paranoia, 
in  dementia.  Some  authors  regard  the  termination  of  these 
psychoses  in  dementia  as  characteristic  of  the  specific  psy- 
chosis. This  view  cannot  be  accepted,  although  it  must  be 
admitted  that  such  an  ending  often  occurs.  Dementia  also 
is  caused  by  diffuse  syphilitic  brain  disease.  Its  patho- 
genesis  is  then  sometimes  characterized  by  the  apparently 
intoxicated  and  semiconscious  condition  described  by 
Heubner. 


180  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

There  is,  moreover,  a  primary  dementia  in  syphilis, 
which  is  not  a  postsyphilitic  dementia,  as  Krause  has  re- 
cently pointed  out.  This  form  of  dementia  is  very  difficult 
to  differentiate  from  paresis,  and  not  infrequently  appears 
as  a  simple  progressive  dementia.  It  manifests  itself  either 
as  a  simple  mental  weakness,  or  develops  more  in  the  form 
of  ethical  defects,  with  a  general  blunting  of  the  sensibili- 
ties and  a  tendency  to  roughness,  lying,  drunkenness,  and 
extravagance.  This  type  of  dementia  in  luetics  may  be 
progressive  or  stationary,  especially  if  specific  therapy 
takes  effect,  and  it  may  finally  disappear,  as  I  have  seen  it 
do.  Nevertheless,  cases  of  complete  recovery  are  the  excep- 
tion rather  than  the  rule.  Usually  defects  in  intelligence 
are  left  behind. 

Korsakoff's  Symptom-complex. — Korsakoff's  symptom- 
complex  has  also  been  observed  (Eoemheld)  in  luetic  pa- 
tients. Typical  cases  of  this  form  of  mental  disturbance, 
where  alcohol  could  be  definitely  excluded,  have  been  re- 
ported by  numerous  observers.  Stransky  has  reported 
such  a  case  in  a  woman  who  had  also  tabes  and  whose  hus- 
band was  affected  with  progressive  paralysis.  In  this 
case  the  mental  disturbance  disappeared. 

One  should  always  keep  in  mind  that  the  most  varied 
forms  of  psychoses  can  occur  in  brain  syphilis,  and  that 
these  forms  do  not  necessarily  mean  an  expression  of 
general  paresis. 

There  is  No  Mental  Disturbance  Characteristic  of  Syphilis. — 
In  regard  to  the  question  as  to  whether  there  is  a  type  of 
mental  disease  which  is  characteristic  of  syphilis,  the  an- 
swer must  be  given  in  the  negative.  As  Oebeke  says,  he 
has  never  recognized  a  pathognomonic  luetic  psychosis,  a 
psychosis  with  characteristic  symptoms  and  a  well-marked 
course,  and  a  favorable  reaction  to  antispecific  therapy. 
On  the  other  hand,  there  is  no  type  of  mental  disturbance, 
from  the  manifold  symptoms  of  a  general  interference  with 
the  nutrition  of  the  brain,  on  through  the  different  forms  of 
the  functional  psychoses,  to  the  psychic  symptoms  which 
are  exhibited  as  a  consequence  of  local  and  diffuse  organic 


NEUROSES  AND  PSYCHOSES  181 

brain  disease,  which  may  not  be  observed  as  the  result  of 
syphilis. 

One  may  say  in  general,  with  reference  to  the  establish- 
ing of  a  relationship  between  lues  and  mental  disease,  if 
other  causes  for  the  psychic  disturbance  are  absent,  such 
as  heredity,  psychic  and  physical  trauma,  alcoholism,  de- 
bilitating bodily  factors,  or  infection  and  intoxication  of 
any  kind,  if  antispecific  treatment  yields  a  more  brilliant 
result,  and  if  the  mental  disturbances  are  accompanied  by 
other  signs  of  syphilis  in  the  body,  then  the  presumption 
is  to  be  regarded  as  greatly  in  favor  of  a  specific  origin. 

It  is  worthy  of  mention  here  that  a  psychosis  may  de- 
velop during  the  administration  of  antispecific  treatment, 
and  that  this  fact,  in  itself,  is  no  proof  of  the  non-specific 
origin  of  the  mental  affection. 


IX 

DEMENTIA  PARALYTICA  AND  SYPHILIS 

Relationship  of  Dementia  Paralytica  to  Syphilis. — At  the 
outset  it  may  be  stated  that  general  paresis  is  not  a  specific 
syphilitic  disease  of  the  brain,  but  that  the  relationship 
existing  between  it  and  syphilis  is  manifold  and  intimate. 
Paresis  claims  a  right  to  particular  attention,  since  it  is 
increasing  in  a  startling  manner. 

Increase  of  Paresis. — According  to  statistics  of  Althaus, 
taken  from  the  insane  hospitals  of  England  during  the 
years  1838-40,  12.6  per  cent,  of  the  patients  admitted  were 
paretics.  On  the  other  hand,  statistics  from  the  same  insti- 
tution during  the  years  1869-91  give  the  average  number  of 
paretics  as  18.1  per  cent.,  while  the  increase  in  all  other 
mental  diseases  was  only  0.2  per  cent.  In  the  insane  hos- 
pital at  Deggendorf,  in  Bavaria,  during  the  years  1867-74 
the  percentage  of  paretics  in  men  was  9.3,  in  women  5.2, 
while  from  1885-90  the  percentage  of  cases  in  men  was  23.2 
and  9.3  in  women.  In  Berlin  34.6  per  cent,  of  the  cases 
were  in  men  and  17.5  per  cent,  in  women;  in  Munich  36.3 
per  cent,  in  men  and  12.2  per  cent,  in  women;  in  Budapest 
36.5  per  cent,  in  men  and  7.5  per  cent,  in  women. 

Earlier  Development  of  Paresis. — An  important  fact  for 
the  clinician  to  keep  in  mind  is  that  paresis,  at  the  present 
time,  may  develop  comparatively  early  in  life.  Cameil,  in 
statistics  collected  from  a  series  of  cases  in  1828,  found 
the  average  age  for  the  appearance  of  the  disease  in  these 
cases  was  44  years,  while  in  the  statistics  of  Regis  and. 
Kaes  the  average  age  was  38. 

The  Increase  among  Women. — The  frequency  of  the  affec- 
tion in  women  has  also  increased.  Formerly,  according  to 
the  admissions  in  the  institutions  for  the  insane,  the  ratio 
of  frequency  between  men  and  women  was  8  to  1.  In  1880 
Reinhardt,  at  Friedrichsberg,  found  the  ratio  to  be  3.2 

182 


DEMENTIA  PARALYTICA  AND  SYPHILIS          183 

to  1 ;  Alzheimer,  in  the  Royal  Psychiatrical  Clinic  in  Munich, 
for  the  years  1894  and  1895,  almost  2  to  1.  In  France  the 
ratio  is  2.4  to  1. 

Other  Causes  of  Paresis. — The  causes  of  this  undoubted 
increase  in  paresis  may  be  attributed  to  the  increase  in  the 
factors  which  are  in  general  injurious  to  the  nervous  sys- 
tem :  the  refinements  of  civilization,  the  increase  in  compe- 
tition along  all  lines,  the  greater  consumption  of  alcohol, 
and  the  pursuit  of  pleasure  of  all  kinds.  Alzheimer  con- 
siders alcoholism  of  first  importance  in  accounting  for  the 
growth  of  paresis  among  the  women  in  Bavaria. 

One  may  also  explain  from  these  etiological  factors  the 
further  facts  that  general  paresis  is  a  disease  which  attacks 
an  individual  during  the  best  years  of  his  life  (between 
thirty-five  and  fifty),  that  it  occurs  more  often  in  the  cities 
than  in  the  country,  and  that  it  seldom  occurs  among  the 
women  of  the  higher  classes. 

Syphilis. — The  honor  of  having  first  discussed  the  rela- 
tionship between  syphilis  and  general  paresis  belongs  to 
Esmarch  and  Jessen.  Both  of  these  observers  reported,  in 
1857,  three  cases  of  paresis  and  syphilis  in  which  they  attrib- 
uted the  paresis  as  due  to  the  syphilis.  iSo  the  discussion 
of  the  question  was  opened,  and  it  was  demonstrated,  as  we 
shall  see  later  in  the  consideration  of  tabes,  that  statistics 
have  not  furnished  a  firm  foundation  for  definite  con- 
clusions, as  the  observations  of  different  authorities  led  to 
different  results. 

Statistics. — It  is  not  necessary  here  to  present  the  great 
mass  of  statistics  on  this  subject.  The  degree  of  their  varia- 
tion has  been  great.  Eickholt,  for  example,  found  a  history 
of  syphilis  in  only  11  per  cent,  of  his  cases  of  general 
paresis,  while,  on  the  other  hand,  Regis  discovered  a  speci- 
fic history  in  94  per  cent,  of  his  cases. 

The  statistics  of  Rieger  are  probably  the  most  compre- 
hensive on  the  subject,  and  permit  one  to  form  as  definite 
an  opinion  as  one  ever  can  from  statistics  alone.  Rieger 
collected  the  statistics  from  eleven  reliable  and  experienced 
observers,  and  ascertained  in  this  way  that  in  1000  cases 
of  paresis,  syphilis  was  present  in  the  history  of  about 


184  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

40  per  cent,  of  the  cases,  and  in  1000  cases  of  patients  with 
other  mental  diseases  syphilis  was  present  in  the  history  in 
about  4  per  cent,  of  the  cases. 

Krafft-Ebing  has  collected  some  other  clinical  observa- 
tions from  the  literature,  which  are  also  pertinent  to  this 
subject. 

Blaschko  found  that  in  Denmark  the  ratio  of  syphilis 
between  men  and  women  was  4.1  to  1 — the  same  ratio  that 
exists  between  male  and  female  paretics ;  also  that  approxi- 
mately the  same  ratio  of  frequency  between  syphilis  in  the 
cities  and  in  the  country  exists  between  paresis  in  the  city 
and  country.  It  has  also  been  proven  that  in  countries 
where  syphilis  is  prevalent,  such  as  Boumania  for  instance, 
paresis  is  also  common ;  and  vice  versa,  in  countries  where 
syphilis  is  rarely  found,  as  in  some  of  the  Swiss  cantons, 
paresis  is  rare. 

On  the  other  hand,  as  is  well  known,  in  Turkey,  where 
syphilis  is  common,  there  is  little  general  paresis.  Krae- 
pelin  and  others  have  proven,  by  their  studies  in  lower 
India,  that  among  the  native  population  paresis  did  not 
occur ;  while  syphilis  was  by  no  means  lacking.  Kraepelin 
explained  the  cause  of  this  as  either  due  to  race  peculiari- 
ties, or  to  harmful  factors  which  rendered  Europeans  less 
resistant  to  the  effects  of  syphilis  on  the  brain  and  cerebral 
vessels  than  the  natives.  The  same  disproportion  exists 
between  syphilis  and  paresis  in  Algiers,  China,  Japan,  and 
Abyssinia. 

Perhaps  the  explanation  of  the  reason  why  paresis 
occurs  so  rarely  in  women  of  the  higher  classes  lies  more 
in  the  fact  that  they  are  not  compelled  to  enter  into  the 
struggle  of  life  than  that  syphilis  rarely  occurs  among  them. 

A  further  fact  which  is  also  true  in  tabes  is  that  certain 
classes  which  are  particularly  exposed  to  venereal  infection, 
as  sailors,  officers,  and  commercial  travellers,  more  often 
develop  paresis;  while,  on  the  other  hand,  the  clergy,  in 
whom  venereal  infection  is  unusually  rare,  very  seldom  are 
affected  with  paresis.  Krafft-Ebing  states  that  in  3000 
cases  of  paresis  in  men  there  was  only  one  Catholic  priest, 
and  that  this  priest  had  been  a  syphilitic. 


DEMENTIA  PARALYTICA  AND  SYPHILIS          185 

Infantile  and  Juvenile  Paresis. — During  the  last  decade  the 
observations  of  the  occurrence  of  paresis  in  children  and 
youthful  persons  has  greatly  increased.  This,  however, 
does  not  mean  that  the  juvenile  type  of  paresis  is  on  the 
increase,  but  rather  that  our  attention,  in  recent  years,  has 
been  more  sharply  drawn  to  this  form  of  the  disease,  as  a 
result  of  which  these  cases  become  easier  to  recognize.  In 
the  numerous  cases  of  infantile  and  juvenile  paresis  re- 
ported the  cases  in  which  syphilis  in  the  parents  was  not 
proven  have  been  very  few. 

The  following  case  is  extremely  pertinent  here: 

A  woman,  twenty-nine  years  old,  came  under  my  care  at 
the  Eppendorf  Hospital  with  general  paresis.  There  was 
not  the  slightest  evidence  that  she  had  ever  had  syphilis, 
she  was  virgo  Intacta,  and  for  the  assumption  of  an  extra- 
genital  infection  nothing  was  found.  The  history  obtained 
from  the  mother  was  as  follows:  The  patient  was  nursed, 
together  with  another  child,  by  the  mother.  This  child 
died  two  months  later  with  syphilis,  after  the  mother  had 
been  infected  with  a  breast  chancre.  From  this  breast 
chancre  the  mother  infected  the  patient.  Her  father  was 
likewise  infected  by  the  mother,  and  now  after  twenty-nine 
years  the  father  is  tabetic,  the  mother  has  symptoms  which 
are  suspicious  of  paresis,  and  our  patient  has  typical 
paresis. 

In  yet  another  category  of  cases  of  infantile  paresis  we 
find  a  combination  of  different  etiological  factors,  such  as 
bad  heredity,  the  use  of  alcohol  in  one  or  both  parents,  and 
the  bringing  up  in  a  bad  social  environment. 

Alzheimer  found  in  cases  of  infantile  and  juvenile 
paresis  syphilis  in  the  history  of  91  per  cent,  of  the  cases, 
Fournier  and  Krafft-Ebing  in  100  per  cent. 

Fournier  has  particularly  brought  out,  in  contradistinc- 
tion to  other  observers,  that  in  his  cases  the  neuropathic 
heredity  was  not  important.  In  112  cases  of  paresis  re- 
ported by  him  he  was  able  to  find  a  poor  nervous  inheritance 
in  only  two  cases. 

Late  Syphilis — Late  Paresis. — As  in  tabes,  so  it  has  also 
b«en  observed  in  paresis  that  after  a  specific  infection 


186  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

acquired  late  in  life,  the  beginning  of  the  paresis  then  occurs 
during  the  advanced  years.  A  further  analogy  exists  in  the 
frequently-observed  cases  of  paresis  and  tabes  in  husband 
and  wife. 

Combination  of  Paresis  and  Syphilitic  Brain  Disease. — As 
in  tabes,  co  also  in  paresis,  along  with  the  paresis  there 
is  sometimes  a  true  specific  disease  of  the  brain.  One  like- 
wise encounters  Heubner's  arteritis  in  combination  with 
paretic  changes.  A  number  of  such  cases  have  been  re- 
ported, especially  cases  of  infantile  paresis  which  have  de- 
veloped from  a  hereditary  specific  basis,  as  in  Heubner's 
case.  In  this  case  paresis  and  genuine  syphilitic  processes 
were  coexistent. 

At  the  present  time  it  is  largely  a  matter  of  personal 
opinion  whether  one  considers  that  the  pathological  changes 
are  caused  by  a  postsyphilitic  toxin,  as  Strumpell  believes, 
or  a  fermentative  poison,  as  Mobius  says.  Burns  and 
Robertson  assume  that  general  paresis  is  caused  by  a  toxin 
which  is  absorbed  into  the  system  from  the  alimentary 
tract. 

Krafft-Ebing's  Experiments. — Krafft-Ebing  has  endeav- 
ored to  settle  the  question  as  to  whether  paresis  is  a  conse- 
quence of  syphilis,  in  an  experimental  manner.  He  selected 
nine  paretics  in  whom  neither  in  the  history  nor  in  the 
physical  examination  was  it  possible  to  obtain  any  evidences 
of  a  past  syphilis.  He  vaccinated  these  patients  both  with 
the  exudate  from  an  initial  lesion  and  the  secretion  from  a 
mucous  patch.  The  patients  were  kept  under  continuous 
observation  for  80  days  and  not  one  of  them  reacted  to  the 
vaccination.  In  other  words,  in  all  of  them  there  existed 
apparently  an  immunity  against  syphilis.  Since  it  is  well 
known  that  immunity  against  syphilis  exists  only  where 
there  is  inherited  syphilis,  or  where  there  has  been  a  pre- 
vious syphilitic  infection,  it  would  seem  to  be  proven  that 
a  former  syphilis  must  have  been  present  in  these  cases. 

While  most  of  the  alienists,  and  especially  those  in  Ger- 
many and  Austria,  assume  a  causal  relationship  between 
syphilis  and  paresis,  this  view  is  by  no  means  universally 
accepted. 


DEMENTIA  PARALYTICA  AND  SYPHILIS          187 

The  existence  of  this  relationship  has  recently  obtained 
further  support  from  the  contributions  of  Neisser,  Wasser- 
mann,  and  Plaut,  in  the  examination  of  the  blood  and  spinal 
fluid.  This  subject  will  be  considered  in  a  special  chap- 
ter. It  is  only  necessary  to  say  here  that  there  is  no 
disease  of  the  nervous  system  in  which  pleocytosis  and  in- 
crease of  globulin  in  the  spinal  fluid  and  the  complement 
deviation  reaction,  both  in  the  blood  and  spinal  fluid,  occur 
so  regularly  as  in  paresis.  Because  of  these  new  and  im- 
portant facts,  the  views  of  the  opponents  of  the  "no  syph- 
ilis, no  paresis"  theory  have  at  the  present  time  only  a 
historical  interest. 

The  Opponents  of  This  Doctrine. — Of  the  opponents 
Nacke  is  one  of  the  most  extreme.  Nacke  shows  from 
his  statistical  researches  that,  by  a  sufficiently  thorough 
examination  in  paretics,  very  frequently  a  severe  neuro- 
pathic inheritance  can  be  demonstrated.  He  was  able 
in  100  paretics  of  his  own  observation  to  find  such  an 
inheritance  in  37  per  cent,  of  the  cases  with  certainty, 
and  believes,  because  of  the  cases  with  imperfect  histories, 
that  45  per  cent,  would  come  nearer  a  fair  estimate. 

Neuropathic  Inheritance. — Under  this  neuropathic  inherit- 
ance he  includes  functional  and  organic  nervous  diseases  of 
all  kinds,  premature  apoplexies,  suicide,  inebriacy,  congeni- 
tally  abnormal  dispositions,  descent  from  very  young  or 
very  old  parents,  and  births  from  intermarriage.  Nacke 
concluded  from  his  researches  that  paresis  does  not  attack 
a  normal  healthy  brain  but  only  one  which  is  congenitally 
weak.  He  attributes  to  syphilis  an  equal  importance  among 
other  causative  factors,  furnishing  the  last  (offence)  for  the 
development  of  the  affection. 

The  great  importance  of  a  neuropathic  inheritance  for 
the  development  of  paresis  has  also  been  emphasized  by 
Raymond,  who,  otherwise,  is  an  absolute  champion  for  the 
existence  of  a  causal  relationship  between  the  two  diseases. 

The  work  of  Matthes  is  well  known.  He  ascertained 
the  fate  of  all  the  luetic  patients  who  had  been  treated  at 
Jena  during  the  last  few  decades.  In  this  way  he  discovered 


188  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

that  only  between  1  and  2  per  cent,  of  all  the  male  luetic 
patients  in  the  course  of  twenty  years  had  developed 
paresis. 

Leredde  sees  in  paresis,  as  in  tabes,  an  affection  which 
bears  a  direct  relationship  to  syphilis,  and,  with  Mobius, 
of  Germany,  believes  that  where  syphilis  cannot  be  demon- 
strated, nevertheless  the  presence  of  paresis  presupposes 
its  past  existence. 

The  majority  of  experienced  observers  will  recall  cases 
of  paresis  from  which  syphilis  with  fairness  may  be  ex- 
cluded. The  following  two  cases  are  representative  of  this 
class. 

The  first  case  was  that  of  a  young  virgin  in  whom  it  was 
impossible  to  demonstrate  any  extragenital  lesion,  and  not 
the  least  suspicion  existed  of  a  congenital  syphilis.  As  a 
result  of  repeated  mental  shocks  she  became  paretic  and 
died  three  years  later  of  this  disease.  The  autopsy  re- 
vealed a  typical  paretic  brain.  There  was  no  evidence  of 
syphilis  present. 

The  other  case  was  a  young  man,  twenty-four  years  old, 
who  consulted  me  on  account  of  severe  symptoms  of  cere- 
bral exhaustion.  He  had  never  had  intercourse,  and  a 
painstaking  thorough  examination  failed  to  reveal  any  evi- 
dence of  an  extragenital  infection.  His  parents  were 
healthy  people.  He  had  naturally,  both  physically  and  men- 
tally, little  resistance.  He  was  unfortunate  in  his  business 
associations,  and  was  compelled  to  work  hard  in  unfavor- 
able surroundings.  An  excited  state  soon  developed  of  a 
paretic  nature,  which  quickly  became  a  progressive  de- 
mentia that  ended  in  death. 

These  two  observations  of  mine  were  made  at  a  time 
when  the  four  reactions  were  unknown,  and  for  this  reason 
can  no  longer  be  regarded  as  valid  arguments  against  the 
relationship  of  syphilis  and  paresis. 

Pathology. — The  pathology  of  paresis  is  a  primary  de- 
generation of  the  nerve  elements,  differing  according  as  to 
whether  the  ganglion  cells  are  first  affected,  as  Alzheimer 
and  Nissl  have  asserted  occurs  in  acute  cases,  or  the  nerve- 
fibres  are  the  first  to  be  involved.  The  supporting  sub- 


DEMENTIA  PARALYTICA  AND  SYPHILIS          189 

stance  in  the  form  of  a  more  or  less  extensive  glia-prolifera- 
tion,  as  well  as  the  blood-vessels,  is  also  affected.  The  arte- 
rial disease,  however,  does  not  present  the  picture  of 
Heubner's  endarteritis. 

The  question  whether  the  disease  of  the  arteries  is  the 
primary  one  in  isolated  cases  has  not  been  definitely  settled, 
but  the  majority  of  the  authorities  do  not,  at  the  present 
time,  so  regard  it.  On  the  other  hand,  it  is  certain  that  dis- 
ease of  the  arteries  plays  a  very  important  part  in  the 
development  of  paresis,  since  by  the  disease  of  the  arterial 
walls  the  intra-  and  extra-adventitial  lymph  spaces  are  dis- 
placed, in  consequence  of  which  a  stasis  is  produced,  and 
this  secondarily  injures  the  nerve  elements,  as  well  as  the 
glia.  Also  the  chemically-changed  waste  products,  which 
are  excreted  from  the  blood-vessels  into  the  lymph-channels, 
may  injure  the  walls  of  the  lymph-passages  and  the  nerve 
elements  as  well,  both  pathologically  and  in  their  function. 

Nissl  and  Alzheimer  have  on  different  occasions  enumer- 
ated the  various  factors  which  distinguish  the  pathology  of 
paresis  from  that  of  diffuse  brain  lues  and  meningo- 
encephalitis. 

It  is  evident  from  these  considerations  that  one  will  find 
a  mixture  of  primary  degenerative,  of  reactive  inflamma- 
tory, and  atrophic  processes  in  the  nerve  elements  and  the 
tissue  of  the  blood-vessels,  and  that  these  processes  are 
variously  represented  according  as  the  clinical  course  is  an 
acute  or  a  chronic  one. 

Recently  Nissl  and  Alzheimer  have  called  attention  to 
the  regular  appearance  of  plasma-cells  in  the  meninges  and 
perivascular  lymph-spaces.  This  finding  has  been  con- 
firmed by  Ernst  Meyer  and  spoken  of  by  Fournier  as  char- 
acteristic of  general  paresis. 

Binswanger  has  attempted,  from  the  pathology  in  de- 
mentia paralytica,  to  differentiate  three  forms:  the 
meningohydrocephalic,  the  hemorrhagic,  and  the  tabo- 
paretic.  He  admits  that  these  distinctions  are  not  sharply 
defined,  and  that  the  most  varied  transitions  occur.  How- 
ever this  may  be,  all  authorities  are  united  on  the  point  that 
the  pathology  of  general  paresis  is  not  the  pathology  of 


190  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

brain  syphilis.  Some  observers,  nevertheless,  regard  the 
degenerative  processes  found  in  paresis  as  a  result  of  syph- 
ilogenetic  disease  of  the  lymph-  and  blood-vessels  and  the 
meninges. 

Antispecific  Treatment. — Whatever  the  conception  one 
may  have  of  the  origin  of  paresis,  it  must  be  admitted  that 
antispecific  treatment  is  not  able  to  cure  it.  Some  few 
authorities,  as  Leredde,  maintain  that  paresis  is  purely 
syphilitic  in  nature  and  that  it  is  curable.  According  to 
Leredde,  paresis  can  be  cured  if  the  treatment  is  intensive 
enough  and  begun  early  enough. 

According  to  my  own  experience  I  am  compelled  to  state 
that  I  do  not  share  Leredde 's  optimism.  Very  often  in  the 
initial  stages  of  paresis  I  have  administered  large  doses  of 
mercury  in  the  form  of  inunctions  and  injections,  and  only 
in  very  rare  instances  has  improvement  occurred. 

The  Consensus  of  Opinion  Concerning  the  Relationship  of 
Paresis  and  Syphilis. — The  preponderance  of  opinion  on  this 
subject  at  the  present  time  may  be  best  summed  up  in  this 
way.  Syphilis  weakens  the  already  more  or  less  predis- 
posed brain  in  its  resistance,  so  that  the  later-appearing 
injurious  factors  are  able  to  influence  it  toward  the  develop- 
ment of  paresis.  Doubtless,  however,  there  are  many  cases 
in  which  other  harmful  factors  are  absent  and  syphilis  is  to 
be  regarded  as  the  only  cause. 

Spielmeyer's  Work. — Since  the  discovery  of  the  Spiro- 
chcete  pallida  one  can  scarcely  discuss  the  subject  of  paresis 
without  thinking  of  Spielmeyer's  work.  Spielmeyer  has 
pointed  out  the  clinical  and  pathological  points  of  resem- 
blance between  the  sleeping  sickness  (trypanosomiasis)  and 
syphilis.  He  found  in  the  brain  of  patients  who  had  died 
of  sleeping  sickness  that  the  capillary  and  precapillary  ves- 
sels and  the  meninges  were  filled  with  plasma-cells.  He 
also  found,  along  with  acute  and  chronic  processes,  a 
marked  increase  of  glia.  He  has  called  attention,  likewise, 
to  the  similarity  between  the  pathology  of  dourine  and  lues. 
Tn  both  affections,  after  the  primary  lesion  a  general 
exanthema  follows,  "with  a  final  condition  of  severe  anaemia 
and  paralysis. 


DEMENTIA  PARALYTICA  AND  SYPHILIS          191 

Mott  found  in  horses  changes  in  the  meninges  which 
resembled  specific  changes,  and  Spielmeyer  found  in  ani- 
mals with  dourine,  infiltrations  which  were  genuine  granu- 
loma,  with  blood-vessels  that  showed  a  proliferation  of  the 
elastica  and  adventitial  infiltration  with  plasma-cells  and 
lymphocytes. 

These  resemblances  between  dourine  and  syphilis  on  the 
one  hand  and  the  sleeping  sickness  and  paresis  on  the  other 
have  a  special  interest  because  of  the  fact  that  Schaudinn 
has  been  able  to  demonstrate  transitional  forms  between 
trypanosomiasis  and  spirochaetes.  One  can  assume  that  the 
relationship  between  these  two  forms  of  germ  life  is  a  very 
close  one. 

Finally,  it  should  be  mentioned  that  Straussler  in  recent 
pathological  examinations  has  demonstrated  the  combina- 
tion of  paresis  and  genuine  syphilitic  processes. 

Differential  Diagnosis. — The  differential  diagnosis  of  de- 
mentia paralytica  is  of  the  greatest  practical  importance. 
It  is  often  difficult  to  distinguish  it  from  a  number  of  brain 
affections. 

Diffuse  Syphilitic  Meningitis. — In  the  first  place,  the  dif- 
fuse meningeal  form  of  syphilis  must  be  differentiated  from 
it.  In  both  affections  the  patients  often  complain,  for  a  long 
time,  of  headache  and  dizziness,  weakness  of  mental  activ- 
ity, and  loss  of  memory.  Transient  paralysis  of  the  cranial 
nerves  and  the  extremities  may  occur  in  both.  Symptoms 
which  speak  for  the  diffuse  meningeal  form  of  syphilis  are 
well-marked  sensitiveness  of  the  skull  to  percussion,  long- 
existing  paralysis  of  the  basilar  cranial  nerves,  which  yield 
to  antispecific  therapy,  and  the  presence  of  disease  of  the 
optic  nerve.  Psychic  disturbances  which  are  either  acute  or 
chronic  in  form  may  be  observed  in  both  conditions ;  in  the 
diffuse  meningitic  type  of  lues,  however,  they  usually  ap- 
pear more  in  the  form  of  a  gradually-increasing  apathy, 
which  may  lead  to  dementia,  while  in  paresis,  frequently 
symptoms  of  irritation  in  the  form  of  psychic  irritability, 
depressing  and  maniacal  delusions,  which  may  develop  to  a 
state  of  maniacal  excitement,  are  observed. 

In  rare  instances  the  poisoning  of  the  brain  by  the  menin- 


192  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

gitic  form  of  lues  may  produce  similar  psychic  anomalies 
to  those  which  are  regarded  as  characteristic  of  paresis. 

The  Arteritic  Form  of  Brain  Syphilis. — The  endarteritic 
form  of  brain  syphilis  manifests  itself  usually  in  hemipare- 
tic  and  paralytic  conditions,  and  the  psychic  disturbances 
appear  more  as  a  clouding  of  the  consciousness,  a  semilucid 
condition,  than  as  symptoms  of  psychic  irritation  and  weak- 
ness. The  endarteritic  form  may,  after  many  years,  end  in 
paresis. 

The  following  is  a  case  of  endarteritis  luetica,  with  later 
an  acute  paresis : 

A  man,  fifty  years  of  age,  fifteen  years  before  had  con- 
tracted lues.  For  three  years  he  had  complained  of  weak- 
ness of  memory  and  inability  to  think,  as  well  as  attacks  of 
dizziness.  Objectively  his  capacity  for  work  was  dimin- 
ished, likewise  his  ability  for  logical  reasoning  and  his 
memory  for  recent  events.  There  was  also  a  slight  disturb- 
ance in  his  articulation,  and  he  had  Argyll-Eobertson  pupils 
on  both  sides.  Six  months  later  he  developed  a  hemiparesis 
on  the  left  side,  which  disappeared  in  two  weeks.  Under 
combined  treatment  of  mercury  and  iodide  the  disturbance 
of  speech  cleared  up  and  the  mental  condition  improved 
so  much  that  his  family  considered  him  entirely  well.  The 
condition  of  the  pupils  remained  unchanged.  He  returned 
to  his  business  and  conducted  it  in  a  wholly  satisfactory 
manner  for  two  years.  Then,  in  order  to  tone  himself  up  a 
little,  he  went  to  a  foreign  water-cure.  Here  he  developed 
insomnia,  was  for  a  few  days  slightly  depressed,  and  then 
suddenly  went  into  a  state  of  wild  hallucination,  delirium 
with  grandiose  ideas,  and  rapid  mental  prostration,  from 
which  he  died  in  four  weeks. 

In  the  differential  diagnosis  between  specific  cerebral 
disease  and  paresis  there  are  many  factors  which  render  the 
question  difficult.  In  the  first  place,  there  is  no  doubt  but 
that  the  so-called  classical  form  of  paresis,  which  begins 
with  maniacal  or  depressive  symptoms  and  quickly  attains 
to  severe  defects  of  the  intelligence  and  memory,  and  causes 
the  dissolution  of  the  entire  psychic  personality  under  the 
picture  of  feeble-minded  ideas  of  grandeur,  and  finally  im- 


DEMENTIA  PARALYTICA  AND  SYPHILIS          193 

becility,  at  the  present  time  is  less  frequent,  and  the  simple 
dementia  form  without  the  classical  stigmata  has  become 
more  frequent. 

Secondly,  the  specific  diffuse  cerebral  syphilis  may  pre- 
sent the  picture  of  a  simple  progressive  dementia,  or  it  may 
exhibit  a  variety  of  forms  and  degrees  of  manic  and  de- 
pressive excitement  for  longer  or  shorter  periods. 

Thirdly,  in  both  diffuse  cerebral  syphilis  and  paresis 
there  occur  apoplectiform  attacks  with  transitory  paralyses. 

The  difficulties  of  a  differential  diagnosis  are  recognized 
by  all  who  have  made  a  special  study  of  paresis  and  brain 
lues.  Oppenheim  states  that  in  two  of  his  cases,  where  he 
made  the  diagnosis  of  paresis,  the  patients  recovered  to 
such  an  extent  after  antispecific  therapy  that  this  diagnosis 
was  no  longer  tenable. 

In  this  connection  it  might  be  again  observed  that  pa- 
resis, with  and  without  antispecific  therapy,  may  show  great 
improvement,  both  in  the  somatic  and  psychic  symptoms, 
and  that  only  those  who  have  known  the  patient  intimately 
are  able  to  detect  the  intellectual  and  moral  delinquencies. 
In  such  cases  one  must  be  prepared  either  for  a  gradual  or 
sudden  further  exacerbation  of  the  disease. 

Is  Recovery  in  Paresis  Possible? — It  had  previously  been 
considered  that  a  genuine  paresis  is  incurable,  but  this  opin- 
ion cannot,  at  the  present  time,  be  unqualifiedly  maintained. 
I  know  from  many  conversations  with  experienced  phys- 
icians that  the  majority  of  them  occasionally  have  observed 
the  recovery  of  some  case  with  the  typical  symptom-com- 
plex of  paresis.  iSchiile  has  reported  a  case  of  undoubted 
paresis  with  recovery  after  an  attack  of  double  pneumonia 
and  severe  suppurative  otitis  media.  This  patient  has  re- 
mained well  twenty  years.  Gaupp  saw  in  Kraepelin's  clinic, 
in  Munich,  cases  of  paresis  with  unusually  long  remissions. 
Halban  has  reported  some  cases  of  paresis  with  recovery. 
In  one  case  the  mental  condition  had  remained  normal 
eleven  years,  in  another  fifteen  years,  after  presenting 
classical  pictures  of  dementia  paralytica.  In  nineteen  years 
I  have  observed  ten  cases  in  which  I  believed  recovery  had 
occurred.  From  these  ten  cases,  four  must  now  be  excluded 

13 


194  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

because  time  has  demonstrated  that  they  were  only  cases  of 
long  remissions.  Of  the  six  remaining  cases,  two  may  be 
enrolled  under  syphilitic  dementia,  which  leaves  four  cases 
of  recovery  in  paresis. 

Because  of  this  great  rarity,  two  of  these  cases  are 
reported  here. 

The  first  case  belonged  to  a  taboparalytic  family.  The 
man  contracted  syphilis,  infected  his  wife,  who,  after  anti- 
specific  treatment,  bore  a  son.  This  son,  when  ten  years  old, 
developed  brain  syphilis,  which  left  behind  it  pupil  anoma- 
lies and  feeble-mindedness.  The  wife  developed  a  station- 
ary tabes.  The  man  himself,  after  a  stage  of  excitement, 
was  admitted  into  a  private  sanatorium,  where  he  was  en- 
rolled as  an  undoubted  case  of  dementia  paralytica.  He 
presented  an  excited  state  with  some  dementia  and  ideas  of 
grandeur.  At  the  same  time  the  light  reaction  of  both 
pupils  was  lost,  there  were  also  slight  difficulties  of  articu- 
lation, and  the  deep  reflexes  of  the  lower  extremities  were 
increased. 

Against  the  advice  of  the  physician,  his  wife  took  him 
home,  where  he  gradually  recovered  so  that  at  the  end  of 
a  year  he  was  able  to  again  take  up  his  business.  He  con- 
tinued his  business  with  good  success  for  six  years.  Then 
one  day  he  suddenly  died  of  heart  trouble.  Of  the  somatic 
symptoms  which  remained  during  these  six  years  there  was 
only  mydriasis  and  loss  of  the  pupil  reaction  to  light. 

A  gentleman,  thirty-seven  years  old,  came  under  my 
observation  during  the  years  of  1901  and  1902.  He  had 
always  been  healthy  and  had  acquired  syphilis  six  years 
previously.  He  had  never  been  a  drinker,  had  a  good  ner- 
vous inheritance  and  had  suffered  no  injury  to  his  head. 
For  the  space  of  a  year  his  articulation  had  been  poor.  Two 
years  before  consulting  me  he  had  severe  epileptic  attacks 
in  which  he  bit  his  tongue.  During  the  last  few  months  his 
speech  became  worse  and  his  memory  poor.  He  was  irri- 
table and  easily  excited  to  anger,  whereas  before  he  had 
been  good-natured  in  disposition. 

Objectively  there  was  weakness  in  the  right  facial, 
tremor  of  the  tongue,  slight  articulatory  disturbances  of  the 


DEMENTIA  PARALYTICA  AND  SYPHILIS          195 

speech,  the  light  reaction  of  the  left  pupil  was  lazy,  and  the 
deep  reflexes  of  the  lower  extremities  were  active.  The  pa- 
tient remained  fourteen  days  in  the  hospital  and  then  with- 
drew from  further  treatment.  A  year  later  he  again  entered 
the  hospital  because  of  severe  headache,  loss  of  memory, 
and  a  state  of  excitement,  During  the  year's  interval  the 
patient  had  taken  up  his  business,  that  of  running  a  butcher 
shop,  but  in  conducting  the  business  he  had  to  be  governed 
by  his  wife,  because  he  was  not  reliable. 

Objectively  there  were  again  found  weakness  of  the 
right  facial,  increased  tendon  reflexes,  a  slight  difficulty 
in  articulation,  myosis  and  loss  of  the  pupil  reaction  to 
light  on  both  sides.  Mentally  there  was  a  weakening  of  the 
intelligence  and  memory.  During  his  six  weeks '  stay  in  the 
hospital  the  dementia  progressed  steadily.  A  course  of 
inunctions  produced  no  change  in  either  the  subjective  or 
objective  symptoms.  He  was  discharged  with  the  diagnosis 
dementia  paralytica.  Six  years  later  I  had  the  opportunity 
of  again  seeing  this  patient,  and  found  him  mentally  abso- 
lutely normal.  I  ascertained  from  his  wife  that  he  had  re- 
mained mentally  weak  for  about  one  year,  that  after  this  he 
gradually  improved,  and  during  the  last  five  years  had  been 
able  to  manage  his  affairs  fully  as  well  as  ever.  Objectively 
the  only  physical  anomalies  left  were  the  myosis  and  loss  of 
the  pupil  reflexes  to  light. 

These  are  cases  whose  enrolment  under  the  diagnosis  of 
dementia  paralytica  no  one  would  have  doubted  if  their 
termination  had  been  the  customary  unfavorable  one.  The 
fact  alone  that  the  outcome  was  favorable,  according  to  my 
opinion,  should  not  change  the  diagnosis.  We  should  con- 
sider it  an  obligation  to  report  all  such  cases  which  come 
under  our  observation  and  possibly  reconstruct  our  present 
opinion  that  all  cases  of  general  paresis  are  incurable. 
Those  who  believe  in  the  absolute  incurability  of  paresis 
will  say  that  such  cases  were  either  cases  of  diffuse  brain 
syphilis  or  that  they  presented  unusually  marked  and  long- 
continued  remissions.  Practically,  however,  these  cases 
should  teach  us  to  be  more  cautious  in  our  prognosis  of 
paresis  than  formerly  we  were  accustomed  to  be. 


196  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

Syphilitic  Pseudoparesis. — Concerning  specific  pseudo- 
paresis,  and  whether  one  is  justified  in  making  a  separate 
entity  of  this  affection,  much  has  been  written.  Fournier 
and  his  pupils  were  the  first  to  describe  this  disease. 

Fournier  says  that  not  infrequently  after  a  luetic  infec- 
tion a  symptom-complex  develops  which  in  the  psychic 
sphere  greatly  resembles  paresis,  with  the  features  of  de- 
mentia strongly  marked.  By  antispecific  therapy  this  con- 
dition disappears  because  the  pathological  basis  of  this 
symptom-complex  is  not  one  of  a  general  process  of  de- 
generation as  in  paresis,  but  is  caused  by  local,  usually 
multiple  and  superficial,  specific  processes  in  the  brain.  It 
is  said  to  be  justifiable  to  diagnose  this  form  of  brain  syph- 
ilis when  the  mental  symptoms  are  combined  with  conditions 
of  hemiparesis,  hemiepilepsy,  tremor  of  the  extremities,  and 
irregular  symptoms  from  the  posterior  columns.  Espe- 
cially useful  in  the  assumption  of  a  pseudoparesis  syph- 
ilitica  are  paralyses  of  the  internal  and  external  eye  mus- 
cles, disease  of  the  optic  nerve,  and  changes  in  the  field  of 
vision. 

These  cases  are  comparatively  frequent  and  their  early 
recognition  is  of  great  importance.  Fournier 's  phrase- 
ology of  pseudoparesis  is,  however,  not  generally  accepted, 
because  these  cases  are  not  cases  of  paresis  but  of  brain 
syphilis. 

Forster  wishes  to  reserve  the  designation  pseudoparesis 
for  those  rare  cases  which  begin  as  classical  paresis,  and 
then  either  become  quiescent  or  completely  recover.  The 
acceptance  of  the  term  pseudoparesis,  according  to  For- 
ster's  limitation,  has  found  the  most  favor  in  Germany. 

The  following  is  a  case  of  pseudoparesis  in  the  sense 
of  Fournier : 

A  laborer,  forty-three  years  old,  had  eighteen  years 
before  his  present  illness  contracted  syphilis.  He  had  taken 
a  number  of  inunction  courses.  Five  years  ago  he  had  been 
treated  in  the  hospital  on  account  of  severe  headache.  The 
headache  yielded  only  to  mercurial  treatment.  Six  weeks 
ago  he  noticed  that  it  was  difficult  for  him  to  articulate, 
his  memory  became  very  poor  and  he  suffered  with  head- 


DEMENTIA  PARALYTICA  AND  SYPHILIS          197 

ache,  which  gradually  increased  in  severity.  At  the  time 
of  his  admittance  into  the  hospital  his  difficulty  in  articula- 
tion was  well  marked,  his  dementia  unmistakable,  and  his 
memory  for  recent  events  was  lost.  Other  prominent  symp- 
toms were  apathy  and  insomnia.  Now  and  then  he  experi- 
enced slight  disturbances  of  consciousness.  He  would  get 
out  of  bed  and  wander  aimlessly  around  the  room,  not  being 
able  to  find  his  way  back  to  the  bed  again.  Two  weeks  later 
the  reaction  of  the  right  pupil  to  light  was  found  to  be  slow 
and  the  left  pupil  was  larger  than  the  right  with  only 
a  slight  response  to  light.  There  was  also  a  beginning  optic 
neuritis  in  both  eyes.  The  tendon  reflexes  in  the  lower  ex- 
tremities were  active.  Ideas  of  grandeur  and  hallucina- 
tion were  not  observed.  From  letters  which  he  wrote  daily 
to  his  wife  the  well-marked  dementia,  incoherence  in 
thought,  and  corticomuscular  disturbances  in  writing  were 
demonstrated.  This  condition  gradually  grew  worse,  and 
only  began  to  improve  after  a  treatment  with  mercury  and 
iodid  had  been  instituted.  Under  this  treatment  the 
patient  almost  completely  recovered  in  the  course  of  a  few 
weeks. 

In  the  differential  diagnosis  between  brain  syphilis  and 
paresis,  if  along  with  the  signs  of  a  diffuse  brain  affection 
there  are  symptoms  of  pressure,  such  as  choked  disc  or  well- 
defined  focal  symptoms,  and  not  the  signs  of  a  general  cor- 
ticomuscular weakness  of  function ;  further,  if  there  exists 
a  combination  of  cerebral  and  spinal  paralysis,  which  ap- 
pears in  irregular  sequence,  and  shows  a  variation  in  inten- 
sity, then  the  clinical  picture  speaks  in  favor  of  a  brain 
syphilis.  In  the  psychic  sphere  the  symptoms  may  be  re- 
garded as  against  the  assumption  of  a  general  paresis  if 
the  changes  of  the  psychic  personality  are  not  the  most 
striking;  but,  if  transient,  disturbances  of  consciousness  or 
depressive  and  maniacal  excitation  and  paralytic  condi- 
tions, along  with  the  preservation  of  the  remaining  mental 
qualities,  are  observed.  Erlenmeyer  points  out,  in  the 
discussion  of  the  differential  diagnosis,  that  in  diffuse  brain 
syphilis  the  delinquencies  of  the  mental  qualities  are  only 
partial. 


198  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

Combination  of  Paresis  and  Brain  Syphilis. — Both  Westphal 
and  Binswanger  have  recently  referred  to  the  clinical  com- 
bination of  dementia  paralytica  and  brain  lues. 

Arteriosclerotic  Brain  Disease  in  Former  Syphilitics. — Not 
infrequently  differential  diagnostic  difficulties  will  present 
themselves  in  patients  with  arteriosclerosis  who  previously 
have  had  syphilis  and  in  whom  transient  cerebral  paralyses 
and  mental  deficiencies  appear.  In  such  cases  the  -question 
presents  itself  whether  we  have  to  deal  with  a  paresis,  a 
specific  endarteritis,  or  with  an  arteriosclerosis  caused  by 
the  previous  syphilis  and  its  consequences.  We  are  justi- 
fied in  assuming  a  general  arteriosclerosis  with  a  positive 
finding  in  the  heart  and  peripheral  arteries,  and  by  the 
presence  of  albuminuria,  when  also  the  somatic  symptoms, 
such  as  the  speech  disturbances,  paresis  of  the  facial  or 
extremities,  are  of  a  more  permanent  nature,  and  when,  as 
a  result  of  the  apoplectiform  originating  paretic  conditions, 
a  greater  or  less  degree  of  diminution  in  intelligence 
develops. 

Diffuse  Arteriosclerotic  Brain  Disease  in  a  Syphilitic. — A 
manufacturer,  forty-four  years  old,  twenty-five  years  ago 
had  syphilis.  He  also  drank  moderately.  There  was  no 
neuropathic  inheritance.  His  business  required  him  to 
work  hard,  both  physically  and  mentally.  As  a  result  of 
business  worry  and  family  care,  he  began  two  years  ago  to 
complain  of  headache,  dizziness,  and  restless  sleep.  His 
family  observed  that  he  was  easily  irritated  and  inclined  to 
sudden  changes  in  humor.  It  became  difficult  for  him  to 
manage  his  business  and  sustained  effort  was  no  longer 
possible. 

The  examination  revealed  a  considerable  degree  of  cen- 
tral and  peripheral  arteriosclerosis,  with  slight  albuminuria 
but  no  casts.  The  psychic  sphere  presented  only  irritability 
and  weakness.  A  stay  of  three  months  in  a  sanatorium 
benefited  him  only  temporarily.  A  few  days  after  he  had 
resumed  his  work  his  dizziness,  headache,  and  insomnia 
returned,  and  he  suddenly  became  confused  and  excited.  A 
sort  of  dazed  condition  developed  in  which  he  had  depres- 
sive delusional  ideas,  and  also  at  times  hallucinations.  He 


DEMENTIA  PARALYTICA  AND  SYPHILIS          199 

was  not  given  at  this  time  antispecific  treatment.  This 
neurasthenic,  irritable,  and  weak  condition  lasted  four 
months,  when  another  attack  similar  to  the  one  described 
above  appeared.  As  a  result  of  this  attack  a  slight  dementia 
developed,  which  manifested  itself  chiefly  in  the  failure  of 
the  patient  to  recognize  his  true  condition  and  an  overesti- 
mation  of  his  capabilities. 

Objectively  there  existed  from  the  beginning  a  slowness 
in  the  response  of  the  left  pupil  to  light  and  an  inequality 
in  the  size  of  the  pupils. 

Immediately  after  his  first  attack  a  suggestion  of  motor 
aphasia  and  a  slight  paresis  of  the  right  facial  appeared. 
During  a  period  of  four  years  this  condition  remained  prac- 
tically unchanged.  The  patient  was  unable  to  pursue  his 
business  and  required  watching  when  he  was  away  from 
home.  A  repetition  of  the  specific  treatment  on  two  differ- 
ent occasions  did  not  produce  any  improvement.  Three 
years  later  the  patient  was  received  into  a  private  sana- 
torium where  he  died. 

The  autopsy  showed  a  diffuse  arteriosclerosis,  particu- 
larly of  the  brain  arteries.  The  brain  macro scopically  did 
not  present  the  picture  of  paresis,  microscopically  there 
was  no  atrophy  of  the  tangential  fibres,  the  increase  of  the 
glia  was  not  such  as  should  correspond  to  a  paresis  which 
had  existed  a  number  of  years,  and  there  was  diffuse 
arterio sclerotic  disease  of  the  small  arteries.  The  spinal 
cord  was  microscopically  intact. 

Recently  Weber,  from  his  observations  in  five  cases,  has 
discussed  in  detail  the  points  of  differential  diagnosis  be- 
tween the  mental  disturbances  in  arteriosclerosis  and  gen- 
eral paresis.  His  cases  were  in  the  fifties  or  somewhat 
earlier,  at  any  rate  in  the  age  of  predilection  for  the  begin- 
ning of  paresis  with  expansive  ideas,  exalted  moods,  and 
motor  restlessness.  At  the  same  time  there  were  some  ob- 
jective symptoms,  especially  relating  to  the  pupils,  present. 
General  paresis  was  excluded  in  Weber's  cases  for  the 
following  reasons : 

First.  The  ideas  of  grandeur  were  more  in  accordance 
with  the  normal  conception  of  the  personality,  better 


200  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

adapted  to  the  patients  calling  and  social  station,  and  the 
patients  attempted  logically  to  prove  these  ideas;  also  they 
were  less  influenced  by  suggestion. 

Second.  In  times  of  great  excitement  and  carelessness 
of  external  behavior,  the  presence  of  mind,  consciousness 
of  personality,  and  knowledge  of  position  remained  intact. 
No  dream-like  states  or  confusion  as  in  paresis  appeared. 

Third.  The  affection  persisted  for  a  long  time  with  the 
intelligence  preserved.  The  memory  and  perception  may 
not  be  disturbed  at  all.  Sometimes  the  patients  have  a  full 
knowledge  of  their  condition. 

Fourth.  Oftentimes  ethical  defects  occupy  the  fore- 
ground of  the  clinical  picture  and  consist  in  a  disappearance 
of  all  altruistic  impulses  towards  the  immediate  family. 
They  are  founded  deeper  than  in  paresis  and  extend,  as  may 
be  ascertained  out  of  the  personal  history,  back  into  the  nor- 
mal days.  The  condition  consists  more  of  a  disappearance 
of  restriction  caused  by  the  disease  than  of  a  character 
metamorphosis.  The  essence  of  the  personality  is  better 
preserved  than  in  paresis. 

Fifth.  The  organic  symptoms  are  in  the  beginning  not 
so  constant  or  so  characteristic  as  they  are  in  most  cases 
of  paresis  with  severe  psychic  disturbances.  Articulatory 
speech  disturbances  are  usually  lacking.  The  pupil  dis- 
turbances are  not  the  simple  loss  of  the  light  reaction  alone, 
but  this  is  combined  with  loss  of  accommodation  as  well. 
When  the  affection  has  existed  a  long  time,  there  are  also 
focal  lesions,  such  as  pareses,  aphasias,  and  disturbances 
in  writing. 

Sixth.  The  duration  of  the  disease  from  its  acute  begin- 
ning is  much  longer  than  in  paresis,  which  is  accompanied 
by  such  severe  psychic  disturbances. 

Seventh.  The  course  is  extremely  variable  but  does  not 
come  to  a  complete  remission.  There  may  be  periods  in 
which  the  patient's  consciousness  is  fairly  clear  and  the 
judgment  good,  without  the  irritable  condition  and  ethical 
defects  entirely  disappearing. 

Eighth.  In  all  the  cases  the  symptoms  originated  from 
an  inherited  tendency,  which  in  addition  to  the  above-men- 


DEMENTIA  PARALYTICA  AND  SYPHILIS          201 

tioned  anomalies  of  character  expressed  itself  in  a  lessened 
resistance  of  the  arterial  system.  Symptoms  of  this  arte- 
rial weakness  appeared  in  the  previous  history  of  the 
patient  in  the  form  of  attacks  of  asthma,  heart  attacks,  and 
angioneurotic  disturbances. 

Ninth.  Along  with  these  endogenous  factors,  exogenous 
agencies  such  as  lues  and  alcoholism  as  accidental  causes  of 
the  disease  appear  in  several  cases. 

Tenth.  The  pathology  is,  according  to  the  autopsy  and 
the  entire  clinical  picture,  a  diffuse  disease  of  numerous 
small  arterial  branches  which  produce  disturbances  in  the 
circulation  and  nutrition  of  the  brain,  and  only  in  later 
stages  lead  to  a  permanent  tissue  change  in  the  form  of 
destruction  of  nerve  elements,  redema  of  the  tissue,  and 
perivascular  glia  proliferation. 

Encephalomalacia  in  Syphilitics. — The  difficulties  which  the 
differential  diagnosis  presents  in  cases  of  encephalomalacia 
in  syphilitics,  who  are  not  manifestly  arteriosclerotic,  is 
well  illustrated  in  the  following  case: 

A  gentleman,  thirty-eight  years  old,  who  had  always 
been  healthy  and  temperate,  contracted  syphilis  when  he 
was  twenty-four  years  old,  and  took  one  course  of  treat- 
ment for  this  disease.  In  the  summer  of  1899  he  complained 
for  the  first  time  of  paraesthesias  in  the  face,  tongue,  and 
arm  and  leg  of  the  right  side.  The  arm  and  leg  also  became 
weak  at  times.  The  examination  of  the  patient  did  not  re- 
veal any  particular  degree  of  arteriosclerosis.  The  only 
somatic  symptoms  were  an  abnormal  activity  of  all  the  deep 
reflexes,  a  slight  limitation  of  the  associated  movements  of 
the  eyes  externally,  and  insufficiency  of  the  left  rectus  in- 
ternus  by  convergence.  Under  mercurial  treatment  the 
attacks  of  paraesthesia  and  weakness  of  the  arm  and  leg 
disappeared  and  the  function  of  the  eye-muscles  became 
normal.  The  tendon  reflexes  continued  abnormally  active. 
The  patient  was  discharged  from  the  hospital  at  the  end  of 
six  weeks.  In  six  months  he  returned  because  of  a  speech 
disturbance  and  a  change  in  disposition.  A  well-marked 
right-sided  hemiparesis  was  now  found  to  be  present,  and 
the  intelligence  was  plainly  weakened.  His  mental  con- 


202  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

dition  was  that  of  a  contented  dement.  At  times  he  became 
excited  and  had  hallucinations  of  hearing  and  sight.  The 
hemiparesis  varied  in  intensity.  Four  months  later  he  had 
an  apoplectic  stroke.  The  right  side  became  spastic  with 
athetoid  movements.  The  aphasia  became  complete.  Gen- 
eral symptoms  which  might  be  expected  to  accompany  a 
brain-tumor  were  absent.  The  patient  developed  decubitus, 
became  dull  and  stuporous,  and  died  a  few  weeks  later. 

The  autopsy  did  not  reveal  macroscopically  any  well- 
marked  arteriosclerosis  of  the  basal  arteries.  There  were 
multiple  small  areas  of  softening  of  the  cortex,  large  gan- 
glia, and  in  various  portions  of  the  medullary  substance, 
more  extensive  on  the  right  side  than  on  the  left.  The 
configuration  and  size  of  the  cortex  was  not  particularly 
changed;  the  pia  was  diffusely  thickened  and  infiltrated. 

The  microscopic  examination  showed  an  extensive 
atheromatous  condition  of  the  small  arteries  and  arterioles. 
The  cells  of  the  cortex  with  the  Nissl  stain  were  in  part 
normal  and  in  part  in  various  stages  of  degeneration.  The 
nerve-fibres  with  the  Weigert-Pal  stain  (areas  from  the 
central  convolutions  were  examined)  were  found  to  be  par- 
tially atrophied,  but  not  with  the  characteristic  atrophy 
of  paresis.  Inflammatory  changes  or  glia  proliferations 
were  not  present  to  any  extent. 

In  this  patient  it  was  difficult  to  tell,  during  life,  whether 
the  case  was  one  of  encephalomalacia,  in  a  very  young  man, 
or  a  rather  unusual  type  of  paresis.  The  autopsy  showed 
it  to  be  one  of  encephalomalacia. 

Cerebral  Neurasthenia. — The  question  of  differential  diag- 
nosis between  cerebral  neurasthenia  and  paresis  also  not 
infrequently  presents  itself.  The  four  reactions,  the  Was- 
sermann  in  the  blood  and  spinal  fluid,  the  globulin,  and 
lymphocytosis  will  aid  materially  in  making  this  distinction. 
Otherwise  the  so-called  "neurasthenic  vorstadium"  of 
paresis  does  not  differ  materially  from  a  true  neurasthenia 
in  a  luetic.  It  might  perhaps  be  said  in  general  that  in 
neurasthenia  the  hypochondriacal  self-analysis  and  self- 
observation  is  more  intense  and  persistent. 

It  is  to  be  remembered  too  that  somatic  symptoms  in 


DEMENTIA  PARALYTICA  AND  SYPHILIS          203 

paresis  may  not  appear  for  a  long  time,  and  on  the  other 
hand  a  neurasthenic  may  present  pupil  anomalies  as  a  result 
of  his  previous  syphilis. 

The  differential  distinction  becomes  especially  difficult 
if,  as  Schiile,  Koppen,  and  Jacobson  have  pointed  out, 
through  arteriosclerotic  changes  in  the  arteries  supplying 
the  anterior  half  of  the  oculomotor  nucleus,  degeneration 
of  the  nucleus  is  produced,  and  as  a  consequence  of  this 
various  pupil  irregularities  appear.  Such  cases  compli- 
cated with  cerebral  neurasthenia  have  been  reported. 

In  general  one  must  recognize  the  fact  that  the  presence 
of  organic  symptoms  in  an  old  luetic  presenting  symptoms 
of  cerebral  neurasthenia  does  not  always  mean  paresis, 
any  more  than  the  absence  of  these  symptoms,  for  a  long 
time,  excludes  the  diagnosis.  In  such  complicated  cases  the 
findings  obtained  by  an  examination  of  the  spinal  fluid  are 
to  be  considered  as  the  surest  means  of  differentiation; 
negative  findings  absolutely  excluding  general  paresis  and 
positive  being  strongly  in  its  favor. 

Chronic  Alcoholism. — Chronic  alcoholism  often  causes 
apathy,  mental  dulness,  abnormal  irritability,  depression 
and  maniacal  moods,  blunting  of  the  moral  sense,  change  in 
the  personality,  and  a  greater  or  less  degree  of  severe 
dementia.  Anomalies  of  the  pupils,  absence  or  increase  in 
the  deep  reflexes,  and  psychomotor  weaknesses  in  the  inner- 
vation  of  the  cranial  nerves  are  also  not  unusual.  The  dif- 
ferential diagnosis  formerly  in  the  beginning  frequently 
had  to  remain  in  doubt  because  of  the  similarity  of  the 
symptoms  and  the  fact  that  alcoholics  are  also  frequently 
luetics ;  at  the  present  time  the  four  reactions  will  clear  up 
the  doubt. 

The  following  case  illustrates  the  degree  of  recovery 
which  can  occur  even  in  severe  alcoholic  psychic  disturb- 
ances : 

A  young  man,  twenty-seven  years  old,  was  admitted  to 
the  hospital  in  a  severe  alcoholic  delirium.  After  the  ter- 
mination of  the  delirium  he  exhibited  the  clinical  picture  of 
a  typical  amentia.  His  power  of  perception  was  totally 
lacking.  His  mood  varied  from  a  state  of  euphoria  to  one 


204  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

of  deep  depression.    He  had  absolutely  no  realization  of  his 
condition.    His  mental  state  was  imbecilic. 

Objectively  there  were  pupil  differences  and  sluggish- 
ness in  their  response  to  light,  deficient  innervation  of  the 
facial  and  tongue  musculature,  and  increased  tendon  re- 
flexes. Three  months  later  his  mind  was  still  very  feeble, 
showing  no  improvement.  After  this,  however,  he  began 
to  improve,  and  in  the  course  of  six  months  his  intelligence 
passed  from  that  of  a  severe  dementia  to  that  of  a  slight 
mental  deficiency.  After  the  expiration  of  a  year  there 
still  existed  some  indifference  and  apathy.  His  memory 
for  the  events  previous  to  his  illness  was  good,  but  for 
recent  events  was  defective.  Later  he  was  able  to  resume 
his  position  in  a  factory  and  keep  it. 

Chronic  alcoholism  may  closely  resemble,  both  in  the 
physical  and  psychic  spheres,  paresis,  and  paresis  may,  on 
the  other  hand,  present  delirious  states  which  are  entirely 
similar  to  an  alcoholic  delirium. 

Brain-tumors. — In  brain-tumors  mental  disturbances  can 
dominate  the  clinical  picture.  In  this  condition  there  is 
usually  found  a  mental  torpor  which  manifests  itself  in  the 
form  of  limitation  in  thought  and  action.  The  patients  act 
and  speak  as  in  a  half-sleep.  They  respond  to  impulse  only 
incompletely  and  for  a,  short  time.  Sometimes  periods  of 
excitement  may  occur.  These  mental  symptoms  are  the 
consequence  of  brain  pressure,  and  with  relief  from  the 
pressure  they  disappear.  Only  by  a  multiplicity  of  tumors 
and  a  well-marked  hydrocephalus  internus  is  the  develop- 
ment of  a  permanent  dementia  possible.  In  such  cases  the 
condition  is  due  to  atrophy  of  the  cortex  by  pressure. 

Syphilis  and  Migraine. — It  is  very  important  to  distin- 
guish between  typical  or  atypical  genuine  migraine  attacks 
and  attacks  of  a  symptomatic  migraine.  Mobius  says  mi- 
graine is  an  inherited  disease  and  begins  in  early  youth. 
In  those  cases  where  its  inherited  nature  cannot  be  demon- 
strated and  the  attacks  do  not  appear  until  later  in  life, 
one  should  be  very  careful  in  making  a  diagnosis  of  mi- 
graine. Not  only  may,  in  disease  of  the  kidneys,  an 
approaching  uraemia  cause  apparent  migraine  attacks,  but 


DEMENTIA  PARALYTICA  AND  SYPHILIS          205 

also  they  may  occur  in  brain-tumors,  both  as  typical  and 
atypical  attacks,  with  visual  and  sensory  aura  which  are 
accompanied  by  paraesthesias  and  aphasia.  In  a  case  re- 
ported by  Abercrombie,  headache  which  resembled  in 
nature  migraine  was  the  only  prodromal  symptom  for  five 
months  of  a  tubercle  of  the  left  hemisphere  of  the  cere- 
bellum. 

Migraine  is  also  a  prodromal  symptom  in  both  tabes 
and  dementia  paralytica.  Charcot  was  the  first  to  call 
attention  to  this  fact.  Oppenheim  observed  migraine  in 
tabetics  very  often.  In  thirty-two  cases  of  tabes  in  women 
he  observed  migraine  in  ten. 

Whether  or  not  the  syphilitic  toxin  can  produce  a  hemi- 
cranial  brain  condition  is  another  question.  We  may  con- 
sider that  the  vasomotor  theory  of  migraine  has  been  aban- 
doned and  that  the  changes  in  function  of  the  arteries  are 
to  be  regarded  as  secondary.  When  in  syphilitics  sympto- 
matic migraine  appears,  it  should  not  be  attributed  to 
arterial  changes  in  the  brain-cortex. 

According  to  my  own  observation  there  would  seem  to  be 
a  causal  relationship  existing  between  a  luetic  intoxication 
and  attacks  of  migraine. 

According  to  a  number  of  cases  reported  by  Halban,  it 
seems  probable  that  migraine  may  sometimes  be  the  only 
late  symptom  in  hereditary  lues. 

The  following  case  is  of  interest  in  this  connection: 

A  woman,  forty  years  old,  the  wife  of  a  bank  director, 
who  had  previously  always  been  healthy,  during  the  last 
year  was  affected  with  a  general  nervousness.  During  the 
last  two  months,  in  addition  to  this  she  had  a  persistent  in- 
somnia, attacks  of  dizziness,  and  severe  headache,  which 
was  most  painful  over  the  parietal  region  and  was  worse  at 
night.  For  a  year  and  a  half  she  had  also  had  frequently- 
appearing  attacks  of  migraine,  which  in  her  earlier  life  she 
had  never  known,  nor  was  there  any  other  case  of  migraine 
in  her  family.  There  were  no  aphasic  or  paretic  disturb- 
ances, also  no  subjective  symptoms  from  the  eyes.  The 
internal  organs  were  normal,  no  arteriosclerosis  was  ob- 
servable, and  the  urine  was  free  from  albumen  and  sugar. 


206  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

The  mind  was  intact.  The  tendon-reflexes  in  both  the  upper 
and  lower  extremities  were  active  without  being  pathologi- 
cally increased.  Sensory  disturbances  were  also  lacking. 
The  pupils  were  abnormally  small  and  the  light  reaction, 
both  direct  and  consensual,  was  slow  and  limited  in  char- 
acter. 

From  the  history  it  was  ascertained  that  her  husband, 
ten  years  before,  had  contracted  syphilis,  for  which  he  had 
been  treated.  Under  antispecific  therapy  the  general  ner- 
vousness, headache,  migraine  attacks,  and  other  subjective 
symptoms  gradually  disappeared.  The  pupil  anomalies 
remained  unchanged. 

In  spite  of  the  permanent  changes  in  the  pupils,  one  is 
not  justified  in  calling  this  case  a  beginning  tabes,  because 
of  the  active  tendon-reflexes  and  the  absence  of  all  other 
symptoms  of  disease  of  the  posterior  columns. 

The  case  is  to  be  regarded  as  one  of  symptomatic  mi- 
graine because  of  the  absence  of  any  hereditary  tendency, 
and  also  because  the  migraine  appeared  rather  late  in  life. 

Attacks  of  ophthalmoplegia  in  severe  attacks  of  idio- 
pathic  migraine  have  occasionally  been  observed.  A  case 
of  acute  complete  ophthalmoplegia,  which  occurred  in  the 
course  of  a  severe  migraine  attack  of  several  days '  duration, 
was  reported  by  Trommer. 

In  such  cases,  when  other  somatic  symptoms  are  lacking, 
one  must  avoid  assuming  a  relationship  with  lues  because 
of  the  ophthalmoplegia.  These  cases  may  be  better  ex- 
plained by  regarding  them  simply  as  an  expression  of 
nuclear  hemorrhage. 


SYPHILIS  OF  THE  SPINAL  CORD 

Rareness  of  Lues  When  Confined  to  the  Spinal  Cord  Alone. — 
The  spinal  cord  is  in  general  less  often  affected  by  syphilis 
than  the  brain.  This  may  be  explained  by  the  fact  that  the 
most  powerful  factors  in  the  etiology  of  diseases  of  the 
nervous  system,  such  as  heredity,  trauma,  infection,  in- 
toxication, as  well  as  psychic  and  intellectual  lesions  in  their 
fullest  sense,  involve  the  brain  more  frequently  than  the 
spinal  cord. 

Fournier  reports  more  than  five  times  as  many  cases 
of  cerebrospinal  syphilis  as  of  the  pure  spinal  type. 

Gebhard  in  eight  years  in  the  large  clinic  at  the  Charite 
saw  only  nine  cases  of  spinal  lues. 

Extension  of  specific,  disease  to  the  spinal  cord  from 
the  vertebral  column  occurs  far  less  often  than  it  does  to 
the  brain  when  the  cranium  is  affected.  Anatomical  reasons 
may  in  a  measure  seem  to  explain  this.  In  the  cranium 
the  periosteum  and  dura  mater  may  be  regarded  as  practi- 
cally one  covering,  while  in  the  spinal  canal  the  vertebral 
periosteum  and  dura  mater  are  separated  by  a  thick  layer 
of  fatty  tissue. 

According  to  Erb's  experience,  specific  disease  of  the 
spinal  cord  occurs  only  one-tenth  as  often  as  tabes. 

In  making  a  diagnosis,  be  exceedingly  careful  in  the 
assumption  of  a  pure  syphilis  of  the  spinal  cord,  and  keep 
in  mind  always  that  the  combination  of  brain  and  spinal 
lues  is  by  far  the  most  frequent. 

Extensive  specific  changes  in  the  brain  may  remain 
latent  during  life,  as  a  case  reported  by  iSiemerling  shows, 
while  on  the  other  hand  latent  syphilis  of  the  spinal  cord 
may  exist  with  well-developed  brain  lues. 

In  a  case  reported  by  Weygandt  clinically  only  brain 
symptoms  were  manifested,  while  pathologically  extensive 
specific  arterial  changes  were  demonstrated  in  the  lumbar 
cord. 

207 


208  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

In  one  of  my  cases  the  clinical  picture  presented  wa,s  that 
of  a  severe  transverse  myelitis  in  the  dorsolumbar  cord. 
There  were  no  symptoms  indicating  disease  of  the  brain  or 
medulla.  At  the  autopsy,  in  addition  to  the  spinal-cord 
affection,  there  was  found  to  be  a  hard  gummatous  infiltra- 
tion at  the  base  of  the  pons  and  in  the  interpeduncular 
space. 

Spinal  Syphilis  May  Appear  Early. — The  old  opinion  that 
spinal  syphilis  appears  only  in  the  later  stages  of  the  dis- 
ease has  been  proven  incorrect.  Gilbert  and  Lion,  from 
the  material  of  44  cases  of  spinal  lues,  were  able  to  show 
that  quite  a  large  proportion  of  this  number  manifested 
their  first  spinal  symptoms  within  the  first  year  after  their 
infection.  They  were  also  able  to  collect  56  cases  out  of  the 
literature  where  the  spinal-cord  affection  developed  within 
the  first  two  years  after  the  date  of  infection. 

Fournier  in  71  cases  of  spinal  lues  found  that  the  dis- 
ease appeared  during  the  first  year  in  8  cases,  the  second 
year  18  cases,  the  third  year  10  cases,  the  fourth  year  10 
cases ;  between  the  fifth  and  tenth  years  17  cases,  and  be- 
tween the  tenth  and  twenty-fifth  years  4  cases. 

Late  Appearance  of  Spinal  Lues. — The  spinal  affection  may 
also,  as  Fournier 's  experience  has  shown,  appear  very  late. 
Williamson  reports  a  case  which  occurred  27  years  after 
the  infection.  From  my  material  one  case  developed  in  the 
twenty- fourth  year.  In  the  24  cases  which  have  come  under 
my  observation,  one-half  of  them  occurred  within  a  period 
of  five  years  after  the  infection.  This  agrees  fairly  well 
with  Oppenheim's  statement  that  one-half  of  the  cases  of 
spinal  lues  occur  within  a  period  of  six  years  from  the  date 
of  the  infection. 

The  Previous  Syphilis  May  Have  Been  Slight  or  Severe. — 
In  some  of  my  cases  the  original  specific  symptoms  were 
insignificant  in  character.  This  was  not  the  rule,  however. 
Gilbert  and  Lion  have  stated  that,  in  contradistinction  to 
brain  lues,  spinal  syphilis  was  more  apt  to  occur  in  the 
severe  types  of  the  disease. 

Men  More  Frequently  Affected  Than  Women. — Men  are 
more  often  affected  with  spinal  syphilis  than  women.  In 


SYPHILIS  OF  THE  SPINAL  CORD 


209 


my  24  cases,  eight  were  in  women,  16  in  men.  From  these 
eight  cases  only  two  of  them  were  from  the  higher  classes. 
One  must  take  into  consideration,  however,  that  syphilis  in 
general  is  of  more  frequent  occurrence  in  men  than  in 
women  and  also  that  the  life  of  men  predisposes  them  more 
to  organic  nervous  disease  than  that  of  women. 

Influence  of  Therapy. — Brasch  states  that,  as  with  the 
brain,  so  also  with  the  spinal  cord,  the  more  thorough  and 
prompt  the  antispecific  therapy,  the  less  liable  it  is  to  be- 
come diseased. 

Influence  of  Age. — What  has  been  said  in  the  chapter  on 
brain  syphilis  in  regard  to  the  effect  of  syphilis  acquired 


FIG.  38. — The  blood  supply  of  the  spinal  cord  in  a  transverse  section. 

late  in  life,  and  also  the  neuropathic  disposition,  applies 
equally  as  well  here. 

The  clinical  case  of  Fournier  illustrates  the  causal  effect 
of  physical  exhaustion  of  the  spinal  functions  in  the  sense 
of  Edinger.  The  case  was  that  of  a  luetic,  who  after  a  long 
bicycle  ride  developed  a  fatal  specific  myelitis. 

The  Peculiarities  of  Spinal  Lues  Are  Due  to  the  Anatomical 
Peculiarities  of  the  Cord. — The  form  of  the  spinal  cord  causes 
some  peculiarities  in  the  pathology  of  luetic  spinal  disease. 
Owing  to  the  smallness  in  the  diameter  of  the  cord,  symp- 
toms of  a  transverse  lesion  easily  occur.  The  tracts  which 
conduct  the  important  sensory  and  motor,  as  well  as  the 
reflex,  functions  are  situated  closely  together  so  that  slight 
lesions  produce  severe  symptoms. 

14 


210  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

While  in  the  brain  the  blood-supply  is  conducted  in 
straight  paths  from  the  heart;  before  entering  the  spinal 
cord  the  blood  channels  bend  at  right  angles  in  order  to 
enter  the  anterior  median  fissure.  On  the  other  hand,  as 
in  the  brain,  so  also  in  the  spinal  cord  the  small  arteries  are 
associated  internally  with  the  pia  and  penetrate  with  it 
into  the  interior  of  the  cord.  This  explains  the  important 
role  played  by  the  arteries  in  spinal  lues. 

The  membranes  of  the  cord  relatively  are  more  fre- 
quently affected  than  the  membranes  of  the  brain. 

The  Pathology. — The  pathology  of  syphilis  of  the  spinal 
cord  may  be  divided  into  that  of  the  coverings  and  of  the 
cord  itself. 

Syphilis  of  the  Vertebras. — The  bony  envelope  is  very 
rarely  affected.  How  rarely  one  may  realize  when  an 
experienced  pathologist  like  Eugen  Fraenkel,  who  has  had 
the  opportunity  of  observing  the  vast  material  in  the  Ham- 
burger City  Hospital  during  the  last  thirty  years,  states 
that  he  has  never  seen  a  case. 

Leyden  says  that  the  diagnosis  of  specific  vertebral 
exostosis  has  been  too  lightly  made.  The  following  case 
illustrates  how  careful  one  should  be  in  making  the  diag- 
nosis from  the  clinical  symptoms.  A  man,  forty  years  of 
age,  had  contracted  a  syphilitic  affection  on  his  chin.  A 
year  later  he  was  taken  sick  with  some  pains  in  his  neck 
and  soon  developed  a  spastic  paraplegia  of  all  four  extremi- 
ties, with  severe  disturbance  of  sensation  and  slight  in- 
volvement of  the  sphincters,  all  the  cerebral  nerves  remain- 
ing intact.  The  sixth,  seventh,  and  eighth  cervical  verte- 
bras were  sensitive  to  pressure;  pain  on  jarring  or  jolting 
also  existed  and  the  limitation  of  motion  common  in  a 
spondylitis  was  present. 

Under  a  mixed  treatment  of  almost  two  years'  duration 
the  symptoms  slowly  disappeared.  Three  years  later  there 
appeared  a  chronic  fungous  inflammation  of  the  right  ankle- 
joint  which  with  surgical  care  and  renewed  antispecific 
therapy  healed.  Again  after  three  years  there  developed  a 
burrowing  abscess  from  a  diseased  rib.  There  was  no  other 
evidence  of  a  tuberculosis.  Two  years  later  the  patient 


SYPHILIS  OF  THE  SPINAL  CORD  211 

died  with  amyloid  degeneration  of  the  abdominal  glands, 
eight  years  after  his  specific  infection.  The  autopsy  showed 
a  thick  indurated  proliferation  in  the  leptomembranes  and 
the  dura  in  the  cervical  region,  which  microscopically  re- 
vealed nothing  of  the  appearance  of  either  syphilis  or  tuber- 
culosis. The  bodies  of  the  sixth  to  the  eighth  cervical 
vertebrae  were  partially  necrosed.  The  caries  had  healed. 
In  the  apex  of  the  right  lung  there  was  a  small  calcified 
tubercular  area.  In  this  case  there  had  in  all  probability 
been  a  specific  meningitis  and  a  tubercular  caries. 

Staub  reports  a  case  of  a  very  slow-developing  deform- 
ity of  the  vertebral  column,  accompanied  by  a  papulosqua- 
mous'  process  of  the  skin  on  the  face  and  a  swelling  of  the 
glands  of  the  neck.  The  success  of  the  antispecific  therapy 
convinced  Staub  of  the  correctness  of  his  diagnosis  because 
not  only  the  exanthema,  but  also  the  enlarged  glands  and 
the  symptoms  depending  on  the  disease  of  the  spinal  verte- 
brae, such  as  neuralgic  pains  and  symptoms  of  spastic  para- 
plegia in  the  lower  extremities,  disappeared.  At  any  rate, 
it  is  well  to  remember  that  syphilis  of  the  spinal  vertebrae 
is  rarely  met  with  and  in  the  pathology  of  spinal  syphilis 
occupies  a  very  unimportant  place. 

Syphilis  of  the  Membranes. — The  membranes  of  the  spinal 
cord  are  usually  affected  together.  The  pia  and  arachnoid 
become  agglutinated  with  spongy,  newly-formed  connective 
tissue  to  the  inner  surface  of  the  dura  on  the  one  hand 
and  with  the  substance  of  the  spinal  cord  on  the  other. 
Isolated  disease  of  both  the  pia  and  dura  may  occur,  how- 
ever. The  meningitis  may  be  diffuse  or  it  may  manifest 
itself  in  small  or  large  circumscribed  areas.  These  areas 
are  usually  multiple.  The  diffuse  form  of  inflammation 
occurs  more  frequently  in  the  dura.  In  the  pia  the  inflam- 
mation is  oftener  localized.  The  caseation,  necrobiosis,  and 
connective-tissue  metamorphosis  of  the  pia  gummata  is  a 
common  occurrence;  all  the  transitional  types  are  found, 
from  the  fresh  acute  purely  inflammatory  infiltrations  of 
the  membranes  to  the  genuine  connective-tissue  indurations 
which  no  longer  present  any  evidences  of  inflammation. 

The  acute  form  of  meningitis  in  syphilis  is  manifested 


212  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

by  diffuse  small-celled  infiltration  of  the  meninges,  which  is 
especially  localized  around  the  blood-vessels,  as  well  as  by 
proliferations  of  new  connective  tissue.  The  inflammatory 
processes  may  surround  the  cord  in  a  ring-like  manner  or 
they  may  be  located  on  the  anterior  or  posterior  surfaces. 


FIGS.  39  and  40. — The  meningitic  proliferation  is  more  strongly  developed  on  the  posterior 
surface  of  the  cord  than  on  the  anterior.  Cone-shaped  proliferations  penetrate  from  the  meninges 
into  the  cord  itself.  (Chronic  specific  spinal  meningitis.)  (Bottiger.) 

Specific  meningitis  is  by  far  the  most  frequently  located 
on  the  posterior  surface  of  the  cord.  The  disease  is  also 
more  extensive  and  more  marked  in  this  location. 

Oppenheim,  Rumpf,  and  Jiirgens  are  of  the  opinion 
that  the  cervical  cord  is  most  often  affected,  while  Grold- 


Fias.  41-47. — Inflammatory  thickening  of  the  meninges.     Extension  of  the  specific  inflam- 
matory infiltrate  into  the  cord,  especially  in  the  posterior  and  lateral  columns. 


flam  and  Eichter,  according  to  their  experience,  consider 
specific  meningitis  shows  a  predilection  for  the  dorsal  and 
dorsolumbar  cord.  A  review  of  the  literature  does  not 
permit  the  recognition  of  any  essential  difference ;  neverthe- 
less the  cases  in  which  the  chief  involvement  is  in  the  lum- 
bar region  are  rare.  Gummatous  disease  occurs  either  in  a 


SYPHILIS  OF  THE  SPINAL  CORD 


213 


diffuse  form  or  as  circumscribed  small  or  large  tumors. 
In  a  case  reported  by  Kosenthal,  the  tumor  was  3  cm.  long 
and  compressed  the  cord  from  the  second  to  the  third  cervi- 
cal roots.  On  the  other  hand  there  may  be  numerous 
gummatous  tumors  of  the  dura  varying  in  size  from  a  grain 
of  rice  to  a  pea.  The  same  regressive  changes  may  occur 
in  the  spinal  meningeal  gummata  as  have  been  described 
as  occurring  in  the  gummata  of  the  brain. 


FIG.  48. — Part  of  a  transverse  section  from  the  dorsal  side  of  the  cord,  with  the  adjacent 
pia  and  the  root  bundles  lying  in  it.  (Siemerling.)  A,  artery;  V,  veins;  R,  spinal  cord;  P,  pia; 
pr,  posterior  roots. 

The  chronic  inflammatory  process  in  the  cases  in  which 
all  the  membranes  are  affected  histologically  has  its  incep- 
tion from  the  arachnoid. 

The  leptomeninges  are  particularly  predisposed  to  dis- 
ease because  of  their  numerous  blood-  and  lymph-vessels. 

The  leptomeningitis  extends  along  the  septum  to  the 
spinal  cord  and  fresh  and  old  small-celled  infiltrations,  with 
or  without  gummata,  push  cone-shaped  into  the  substance 
of  the  cord. 

There  is  no  doubt,  although  of  rare  occurrence,  but  that 
a  primary  independent  pachymeningitis,  originating  on  the 


214 


SYPHILIS  AND  THE  NERVOUS  SYSTEM 


one  hand  from  the  spinal  vertebrae  and  on  the  other  from 
the  pia  and  arachnoid,  sometimes  occurs. 

Disease  of  the  Blood-vessels. — A  not  inconsiderable  role  is 
played  by  the  disease  of  the  blood-vessels.  Naturally  the 
same  conditions  are  presented  which  have  been  described 
in  the  brain.  We  know  now  that  the  veins  are  rarely  normal 
and  that  along  with  the  stenosing  and  obliterating  endo-, 
meso-,  and  periarteritis  an  endo-  and  periphlebitis  is  almost 
always  a  regular  accompaniment  and  often  produces  veno- 
sclerosis.  In  a  case  of  acute  myelitis  Lamy  found  patho- 


Fia.  49. — Thrombosis  of  diseased  vessels,  with  secondary  hemorrhages.     (Williamson.) 

logical  changes  only  in  the  veins.  The  same  observer  also 
reports  a  case  of  meningomyelitis  in  which  the  veins  alone 
were  affected.  Such  cases  cause  one  to  think,  as  Bieder  has 
shown  as  probably  true  in  the  neighborhood  of  the  primary 
lesion,  that  also  in  the  nervous  system  the  veins  are  the 
first  to  become  involved. 

The  Cord  Itself  is  Not  Primarily  Affected. — The  substance 
of  the  spinal  cord  does  not  become  primarily  affected  in 
syphilis.  The  parenchyma  of  the  cord  becomes  involved 
secondarily  and  in  various  ways.  It  may  be  affected  by  a 
meningitic  process  in  the  form  of  a  circular  sclerosis  on 
the  surface  of  the  cord  or  it  may  become  diseased  trans- 


SYPHILIS  OF  THE  SPINAL  CORD 


215 


versely  along  the  entering  proliferating  pia  septum.  The 
nerve-fibres  are  then  compressed  by  the  chronic  inflamma- 
tory tissue;  also  by  compression,  stasis  in  the  blood-  and 
lymph-channels  is  gradually  produced  with  its  conse- 
quences. The  atrophy  of  the  nerve-tissue  is  followed  by  an 
increase  in  the  neurologia. 

Chronic  Myelitis. — The  meningomyelitis  is  the  most  fre- 
quent form  of  manifestation  of  lues  in  the  spinal  cord.  This 
fact  is  especially  important  for  an  understanding  of  the 
clinical  picture  of  spinal  specific  disease.  Above  and  below 
the  degenerated  areas  sclerotic  changes  occur — secondary 


FIG.  50. — Intraspinal  gummata  with  surrounding  meningitis.     (Williamson.) 

degeneration.  When  the  products  of  inflammation  con- 
strict the  posterior  roots  and  thus  cause  degeneration,  then 
there  results  ascending  intramedullary  degeneration. 

Gummata  in  the  Substance  of  the  Cord. — Gummata  multi- 
ple or  solitary  may  also  occur  in  the  spinal  cord,  either  in 
the  white  or  gray  substance;  in  the  neighborhood  of  the 
gummata,  softening  of  the  substance  of  the  cord  may  occur, 
due  either  to  disease  of  the  blood-vessels  or  to  the  dele- 
terious effect  of  the  specific  toxin  on  the  nerve-tissue. 

Disease  of  the  Gray  Substance. — The  anterior  gray  sub- 
stance is  not  infrequently  affected,  in  the  form  of  changes 
in  the  ganglion-cells,  which  are  swollen,  vacuolized,  with- 
out nuclei,  and  with  their  processes  destroyed.  In  other 
cases  there  are  hyperaemia  and  hemorrhages. 


216 

Transverse  Myelitis. — All  parts  of  the  cord  may  become 
diseased  transversely  in  the  form  of  small  foci  which  were 
formerly  classified  as  myelitis  but  at  the  present  time  are 
more  correctly  regarded  as  vascular  softenings.  If  several 
of  the  foci  are  adjoining,  then  there  is  presented,  as  in  the 
case  with  a  large  focus,  the  picture  of  myelitis  transversa. 
This  process  may  have  an  acute  beginning,  then  one  finds 
microscopically  the  picture  of  simple  necrosis. 

The  White  Substance  More  Often  Affected  Than  the  Gray. — 
The  white  substance  is  much  more  frequently  affected  than 
the  gray,  and  the  posterior  and  lateral  columns  are  oftener 
involved  than  the  other  parts  of  the  white  matter. 

The  question  as  to  whether  myelitis  or  meningitis  is  the 
primary  factor  in  the  pathology  is  an  important  one.  We 
know  that  in  severe  disease  of  the  membranes,  especially 
of  the  pia,  the  white  substance  shows  a  peripheral  sclerosis, 
which  at  the  points  where  the  pia  is  adherent  is  the  most 
marked.  Signs  of  primary  disease  of  the  cord  itself  are 
manifested  by  simple  foci  and  extensive  softenings  and 
cavity  formations  in  the  white  substance. 

Root  Neuritis. — The  roots  may  become  diseased  in  sev- 
eral ways  (Buttersack,  Kahler).  They  may  be  compressed 
by  the  specifically  inflamed  pia,  the  inflammation  may  ex- 
tend from  the  pia  into  the  roots  themselves  and  thus  injure 
the  individual  nerve-fibres  secondarily.  Proliferations  may 
also  originate  from  the  specifically  diseased  vessels,  the 
disease  of  the  roots  may  consist  entirely  in  an  affection 
of  the  vessels,  which  can  take  on  either  the  character  of 
Heubner's  arteritis  or  an  inflammatory  character,  and 
finally,  in  addition  to  the  forms  of  involvement  just  men- 
tioned, gummata  may  become  localized  in  the  roots. 

It  is  worthy  of  mention  that  up  to  the  present  time  where 
the  roots  have  been  affected,  in  severe  disease,  they  have 
never  been  entirely  destroyed,  but  here  and  there  individ- 
ual fibres  have  been  found  preserved. 

Very  often  a  disproportion  exists  between  the  disease  of 
the  spinal  roots  and  the  cord  itself,  but  no  case  has  ever 
been  reported  of  isolated  disease  of  either  the  roots  or  cord. 


SYPHILIS  OF  THE  SPINAL  CORD 


217 


Fia.  51. 


FIG.  52. 


Fia.  53. 


218  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

System  Disease. — The  discussion  concerning  the  so-called 
system  diseases  of  the  cord,  the  systemic  degenerations  of 
the  posterior  and  lateral  columns  and  the  anterior  horns, 
has  received  considerable  attention  in  the  literature  of 
syphilis  of  the  spinal  cord.  Combined  disease  of  the  pos- 
terior and  lateral  columns  in  a  large  number  of  cases  is 
regarded  as  the  result  of  syphilis.  In  these  cases  the 
process  is  undoubtedly  due  to  post-  or  metasyphilitic  disease 
and  not  to  the  causative  factor  of  lues.  We  agree  with 
Striimpell,  who  has  stated  that,  as  a  consequence  of  the 
specific  process,  a' chemical  poison  is  created  which  exercises 
a  selective  action  upon  the  system  tracts  of  similar  func- 
tion. A  genuine  specific  affection  causes  no  system  involve- 
ment, but  portions  of  systems  are  often  affected  in  irregu- 
lar form.  In  the  majority  of  instances  the  system  affection 
is  only  simulated  and  in  reality  there  exists  a  peripheral 
sclerosis  as  a  result  of  insufficient  nutrition  because  of  dis- 
ease in  the  meningeal  vessels.  In  these  cases  the  central 
regions  of  the  white  and  gray  substance  remain  intact.  The 
peripheral  sclerosis  can  produce  ascending  and  descending 
degeneration  and  thus  resemble  system  disease.  In  other 
cases  the  development  of  pseudosystem  disease  depends 
upon  the  topographical  relations  of  the  affection  in  the 
course  of  a  meningomyelitis  (pseudotabes  syphilitica) ,  as 
observations  of  Oppenheim,  Eisenlohr,  and  Ewald  have 
shown. 

A  large  number  of  observations  (Dinkier,  Minor,  Kuli, 
Fr.  Pick,  Nonne)  have  demonstrated  that,  with  the  classi- 
cal parasyphilitic  disease,  tabes,  a  true  specific  meningitis 
may  occur. 

Erb,  in  an  exceedingly  interesting  and  stimulating  study 
called  '  *  Observations  Conc&riung  the  Pathology  of  Syphilis 
of  the  Central  Nervous  System, ' '  expresses  the  opinion  that 
the  apparently  indifferent,  primary,  parenchymatous  de- 
generations (atrophy,  sclerosis,  system  disease)  are  just 
as  much  the  result  of  syphilis  as  the  so-called  gummatous 
specific  lesions  and  the  diseases  of  the  blood-vessels.  These 
atrophies  and  degenerations  should,  with  the  same  right  as 


SYPHILIS  OF  THE  SPINAL  CORD  219 

specific  gummatous  lesions,  be  regarded  as  syphilogenetic 
in  origin  if  the  same  measure  is  applied  to  both.  It  is  in  this 
sense  that  Erb  considers  the  system  disease,  tabes,  and  the 
combined  system  disease,  as  it  has  been  found  in  individ- 
ual cases,  developing  from  a  specific  spinal  paralysis,  as 
syphilitic. 

The  Pathology  of  Spinal  Syphilis  in  Itself  Not  Character- 
istic.— In  conclusion  the  question  naturally  arises  as  to 
whether  all  of  the  pathological  processes  or  a  part  of  them 
are  characteristic  of  syphilis.  With  the  exception  of  the 
gummatous  diseases,  this  question  must  be  answered  in 
the  negative,  and  it  must  further  be  admitted  that  all  of 
the  individual  pathological  findings  in  syphilis  of  the  spinal 
cord  have  analogies  in  non-syphilitic  processes. 

The  residual  of  an  acute  meningitis  in  syphilis  cannot  be 
differentiated  from  other  forms  of  meningitis  of  different 
origin.  All  types  of  meningitis  may  terminate  in  fibrous 
induration.  Even  in  a  gummatous  growth  the  microscopic 
examination  proves  only  that  the  tumor  can  be  of  specific 
origin.  The  combination  of  the  different  findings  is,  how- 
ever, fairly  characteristic  of  nerve  syphilis.  If  one  finds 
gummatous  nodules  in  the  inflamed  meninges  and  spinal 
cord,  in  addition  to  endarteritis  and  endophlebitis,  also 
softenings  and  sclerosis,  either  circumscribed  or  diffuse, 
one  may  assume  with  considerable  assurance  a  specific 
etiology. 

Recent  observations,  however,  concerning  the  manifes- 
tations of  tuberculosis  of  the  cord  have  taken  away  some  of 
the  certainty  from  this  last  assumption. 

iSchamschin,  from  extensive  observations  in  tuberculosis 
of  the  central  nervous  system,  has  described  pathological 
findings  which  in  multiplicity,  in  the  meninges,  parenchyma 
of  the  cord  and  in  the  blood-vessels,  equalled  those  of 
syphilis. 

Confirmation  of  the  pathological  diagnosis,  if  sought  for 
in  the  other  internal  organs,  will  usually  be  discovered. 
In  spite  of  the  absence  of  a  single  characteristic  finding, 
however,  Wieting  is  right  when  he  says  that,  in  the  great 


220  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

majority  of  cases  of  meningomyelitis,  syphilis  may  be 
assumed  as  the  cause.  As  has  been  said  with  reference  to 
brain  syphilis,  so  here  it  is  also  true,  that  a  careful  con- 
sideration of  the  various  factors,  the  relationship  and  con- 
dition of  the  other  internal  organs,  and  last  but  not  least 
the  clinical  course,  will  in  the  majority  of  instances  enable 
one  to  make  a  positive  diagnosis. 

Symptomatology,  Multiplicity  of  Symptoms. — This  review 
of  the  pathology  which  syphilis  of  the  cord  produces  will 
aid  us  to  a  certain  extent  in  a  better  understanding  of  the 
great  variety  of  symptoms  which  spinal  lues  may  present. 
Accordingly  as  the  pathological  changes  are  most  marked 
in  the  meninges  or  in  the  cord  itself,  we  have  meningeal 
or  cord  symptoms  predominating. 

The  Meningeal  Symptom-complex. — The  general  meningeal 
symptoms  are  essentially  the  same  as  in  other  forms  of 
meningitis,  pains  in  the  neck,  between  the  shoulders  or  in 
the  back,  parassthesias,  painful  sensations  and  severe  attacks 
of  pain  which  radiate  to  the  upper  or  lower  extremities 
and  the  upper  and  lower  portions  of  the  body;  objectively, 
hyperaesthesia  and  diffuse  sensitiveness  to  pressure  in  the 
regions  in  which  the  pains  exist.  Along  with  these  sensory 
irritative  symptoms  there  appear  also  symptoms  of  motor 
irritation  in  the  form  of  tension  in  the  musculature  which 
may  increase  to  contractions.  The  tendon-  and  skin-reflexes 
are  increased.  In  almost  all  the  cases  one  finds  a  rigidity 
of  the  back  combined  with  a  localized  or,  what  is  more  fre- 
quent, a  diffuse  tenderness  to  percussion  of  the  vertebral 
column,  as  well  as  a  painfulness  upon  movement.  The 
French  observers  following  Charcot  have  emphasized  the 
point  that  the  pain  at  night  is  more  severe  than  during  the 
day.  In  the  cases  which  I  have  observed,  the  pain  has  not 
been  particularly  worse  at  night  and  I  have  been  unable  to 
find  anything  in  the  German  or  English  literature  to  sub- 
stantiate this  contention.  Severe  paralysis  with  atrophy 
of  the  muscles  and  changes  in  the  electrical  response  of  the 
muscles  affected  are  evidence  that  the  spinal  roots  second- 
arily, because  of  the  meningeal  inflammation,  have  become 
involved.  Naturally  one  encounters  here  temporary  transi- 


SYPHILIS  OF  THE  SPINAL  CORD  221 

tional  symptoms,  since  a  severe  hyperaemia  with  extravasa- 
tion can  exercise  a  slight  compression  upon  the  peripheral 
parts  of  the  cord  and  spinal  roots.  Eeglonal  hyperassthesias 
and  anaesthesias,  vasomotor  paralyses  and  symptoms  of 
irritation  with  some  dysuria  may  be  enrolled  under  the 
meningitic  symptoms.  Slight  paretic  conditions  of  the  ex- 
tremities or  a  part  of  an  extremity  also  belong  to  the  typical 
picture  of  a  meningitis.  Not  infrequently  the  paresis  is 
only  a  pseudo  one,  since  the  patient  through  fear  of  attacks 
of  pain  will  not  move  the  extremities. 

Meningeal  Irritation. — Lang  has  especially  called  atten- 
tion to  the  meningeal  irritation  in  the  beginning  of  the  sec- 
ondary period  of  syphilis ;  the  backache,  paraesthesias  in  the 
upper  and  lower  extremities,  weakness  and  a  feeling  of 
tiredness  in  the  arms  and  limbs,  slight  irritation  of  the 
bladder,  active  skin-  and  tendon-reflexes  he  regards,  as  do 
also  Jarisch  and  Finger,  as  the  manifestation  of  an  inflam- 
matory congestion  of  the  blood-vessels  of  the  spinal 
meninges. 

Fournier  has  also  frequently  observed  in  the  beginning 
of  the  secondary  period  a  regional  symmetrical  analgesia, 
or  a  disturbance  in  the  temperature  sense  and  sense  of 
touch,  which  he  ascribes  to  the  same  cause.  Schnabel  has 
often  seen,  as  an  objective  proof  of  this  hyperaemic  condi- 
tion in  the  inception  of  the  eruptive  stage,  a  hyperaemia  in 
the  background  of  the  eye. 

Friedman  has  described  as  objective  symptoms  the 
regional  analgesia  and  hyperaesthesia,  anomalies  of  the 
tendon-reflexes,  localized  pareses,  and  amyotrophies.  He 
calls  these  symptoms  "complications  of  spinal  irritation  in 
luetics."  A  pathological  finding  has  not  been  reported, 
but  without  doubt  a  search  for  such  lesions  in  the  autopsies 
of  syphilitics  who  have  shown  irritative  meningeal  symp- 
toms of  a  sensory  nature  would  often  lead  to  a  positive 
finding  and  to  a  more  correct  understanding  of  the  existing 
condition. 

Spinal  Meningitis. — These  same  symptoms  are  also  en- 
countered in  the  prodromal  stages  of  myelitis  or  meningo- 
myelitis.  Here  they  are  more  severe.  A  still  better  proof 


222  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

of  the  meningeal  irritation  after  specific  infection  is  found 
in  the  lymphocytosis  and  increase  of  the  globulin  constit- 
uents of  the  spinal  fluid. 

Our  knowledge  of  the  pathology  of  an  actual  meningitis, 
the  spinal  cord  remaining  unaffected,  is  naturally  limited, 
for  it  is  in  such  cases  that  timely  treatment  causes  a  disap- 
pearance of  the  process  and  the  recovery  of  the  patient. 
The  following  case  must  be  regarded  as  one  of  specific 
spinal  meningitis : 

A  man,  twenty-three  years  old,  developed  a  chancre  on 
his  penis  which  healed  under  local  treatment.  Two  months 
later  an  eruption  appeared.  Two  weeks  after  the  disap- 
pearance of  this  eruption  he  complained  of  lancinating  pains 
in  both  limbs,  especially  in  the  right  and  in  the  lower  part 
of  the  spine.  Now  and  then  he  had  paraesthesias  in  the 
right  leg  and  also  a  slight  dysuria.  The  pains  were  about 
the  same  during  the  day  as  they  were  at  night,  varying  in 
intensity  but  usually  severe.  Stiffness  of  the  back  was 
complained  of.  The  right  lower  limb  was  somewhat  weaker 
than  the  left.  Both  the  limbs  were  hypersensitive  to  percus- 
sion, the  right  more  so  than  the  left.  The  same  condition 
existed  for  pin-pricks;  otherwise  sensation  was  normal. 
Both  the  tendon-  and  skin-reflexes  were  active.  Passive 
motion  produced  no  tenseness  in  the  muscles.  The  entire 
spinal  column  was  held  somewhat  rigid  and  in  the  lumbar 
region  was  sensitive  to  both  pressure  and  percussion.  Ker- 
nig's  symptom  was  present.  The  cranial  nerves  were  not 
involved  and  oculopupillary  symptoms  were  absent.  Mer- 
curial inunctions  were  administered,  and  after  the  fourth 
inunction  the  patient  said  his  pains  were  better  and  after 
the  tenth  they  had  entirely  disappeared.  His  other  symp- 
toms likewise  disappeared. 

In  the  following  case,  in  addition  to  the  meningitis,  the 
cord  was  also  slightly  involved : 

A  young  man,  twenty-three  years  old,  had  two  years 
before  contracted  syphilis.  Six  weeks  before  I  saw  him  he 
was  seized  with  girdle-pains  under  the  costal  arch,  paraes- 
thesias in  the  feet  and  legs,  and  a  paresis  of  the  bladder. 
These  symptoms  were  followed  by  a  weakness  in  the  lower 


SYPHILIS  OF  THE  SPINAL  CORD  223 

extremities  which  manifested  itself  in  a  difficulty  in  stand- 
ing and  walking;  a  motor  weakness  was  found  in  the  ele- 
vators of  the  hips,  extensors  of  the  thighs,  and  the  adduc- 
tors. The  patellar  and  Achilles  reflexes  were  active  and 
Babinski's  and  Oppenheim's  phenomena  existed  on  both 
sides.  All  these  symptoms  responded  quickly  to  antispe- 
cific  treatment. 

These  cases  illustrate  well  the  clinical  picture  of  spinal 
meningitis.  They  also  demonstrate  its  quick  response  to 
antispecific  therapy  and  its  excellent  prognosis  so  long  as 
irreparable  secondary  changes  in  the  cord  have  not  occurred. 
They  show  further  that  specific  spinal  meningitis  very  often 
appears  quite  early  in  the  course  of  a  specific  infection. 
Both  Oppenheim  and  Siemerling  have  laid  particular  stress 
on  the  changing  behavior  of  the  patellar  reflexes.  These  re- 
flexes may  be  active  one  day,  weak  the  next,  and  several 
days  later  wanting  entirely,  only  at  a  still  later  time  to  reap- 
pear. The  variation  in  swelling  of  the  specific  infiltrated 
tissue  which  compresses  the  fibres  which  are  responsible  for 
the  patellar  reflexes  is  the  explanation  given  for  this. 


XI 
MENINGOMYELITIS 

Meningomyelitis  the  Most  Frequent  Form  of  Spinal  Syphilis. 
— Meningomyelitis  is  the  most  frequent  form  of  spinal-cord 
syphilis.  We  have  already  learned  in  what  way  the  spinal 
meninges  become  affected,  and  have  also  seen  how  the  cord 
itself  may  become  involved  by  either  acute,  subacute,  or 
chronic  forms  of  meningitis.  We  have  seen  how  the  soften- 
ings, or  myelitis  as  it  was  formerly  called,  may  involve  the 
entire  cord  transversely  in  a  single  lesion,  and  how  the 
lesions  may  also  be  very  numerous  and  disseminated. 

Arterial  Distribution. — Through  the  labors  of  Adamkie- 
wicz  and  Kadyi  we  know  that  the  anterior  gray  matter  as 
well  as  the  adjoining  white  substance  is  richly  supplied  by 
arteries  which  radiate  from  the  periphery  into  the  interior 
of  the  cord.  The  posterior  gray  matter  and  posterior 
columns  are  also  well  supplied  with  their  own  arterial 
system.  These  anatomical  conditions,  together  with  the 
fact  that  the  various  blood-vessels,  arteries,  veins,  and 
capillaries  are  so  exceedingly  prone  to  disease,  will  explain 
in  advance  the  frequency  of  the  vascular  forms  of  specific 
spinal-cord  affections. 

The  Clinical  Symptoms  Depend  Upon  the  Location  of  the 
Arterial  Disease. — It  will  therefore  be  easy  to  understand,  if 
in  a  gradually  developing  arterial  disease  no  region  is  spared, 
why  the  clinical  picture  presented  is  one  of  chronic  trans- 
verse myelitis,  when  the  vessels  in  the  anterior  and  lateral 
columns  are  most  affected,  why  the  picture  of  an  incomplete 
myelitis  presents  itself,  or  when  the  arteries  supplying  the 
posterior  columns  are  the  most  affected,  why  the  symptoms 
of  disease  of  the  posterior  columns  appear.  It  will  further 
become  plain  that  through  a  combination  of  the  processes 
the  symptoms  exhibited  may  be  manifold  in  character,  de- 
pending upon  whether  the  disease  of  the  vessels  extends 
rapidly  and  acutely  in  the  different  cord  areas,  or  whether 
it  encroaches  slowly  and  chronically. 

224 


MENINGOMYELITIS  225 

The  fact  that  both  the  anterior  and  posterior  roots  have 
their  own  arterial  branches  makes  it  possible  for  the  de- 
velopment of  root  symptoms  through  localized  arterial 
disease. 

The  multiple  character  of  meningomyelitis  cannot  be 
emphasized  too  strongly.  The  picture  of  a  compression 
myelitis  may  appear  if  the  meningeal  exudate  is  extensive 
enough  and  slowly  compresses  the  cord  at  a  given  spot,  the 
epidural  and  epipial  proliferations  also  disturb  through 
pressure  the  circulation  in  the  spinal  lymph  system  and 
by  compression  of  the  larger  vessels  create  a  general  trans- 
verse ischemia  and  by  a  shutting  off  of  the  circulation 
of  lymph  a  more  or  less  extensive  oedema  longitudinally 
of  the  cord. 


FIG.  54. — The  dotted  area  is  supplied  by  the  anterior  spinal  artery,  the  lighter  area  sur- 
rounding it  by  branches  from  it  on  the  one  hand  and  also  on  the  other  by  branches  from  the  vasa 
corona ;  the  darker  peripheral  area  is  supplied  by  the  peripheral  arteries  of  the  cord.  (Williamson.) 

If  finally  large  foci  of  softening  or  sclerotic  areas  de- 
velop, then  secondary  degeneration  of  the  ascending  and 
descending  type  appears.  Tumor-like  conical  growths 
which  extend  from  the  meninges  into  the  substance  of  the 
cord  compressing  and  destroying  the  nerve-tissue  also  merit 
consideration.  This  newly-formed  gummatous  tissue  can 
penetrate  into  the  cord  naturally  from  different  points  and 
involve  different  areas,  thus  producing  a  multitude  of  dif- 
ferent symptoms.  Fig.  55  from  Homen's  work  illustrates 
this. 

The  meningitis  is  usually  the  most  severe  in  the  dorsal 
part  since  here  the  spinal  cord  has  the  least  resistance. 
Consequently  myelitis  in  the  majority  of  cases  manifests 

15 


226  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

itself  as  a  dorsal  myelitis.  The  meningitic  symptoms  are 
the  same  as  have  been  described  in  the  previous  chapter. 
They  may  be  very  severe  and  cause  considerable  suffering 
for  a  long  time.  They  exist  for  days,  weeks  and  months  as 
premonitory  symptoms  alone,  indicating  the  gradual  de- 
velopment of  the  disease.  In  other  cases  they  may  be  slight 
and  for  this  reason  not  paid  much  attention  to  by  the  patient 


8 


FIQ.  55. — Syphilitic  spinal  meningitis.     (Homdn.)     Dorsal  myelitis. 

who  complains  of  some  stiffness  and  tiredness  in  the  back, 
drawing  in  the  limbs,  and  rheumatism  in  the  arms.  Then 
the  disease  progresses  further  with  para3sthesias,  and  finally 
a  subjective  feeling  of  weakness  in  the  lower  extremities 
ushers  in  the  myelitis.  The  motor  weakness  may  exist  as 
a  paresis  or  it  may  increase  to  a  complete  paraplegia  which 
is  usually  spastic  in  character. 

Sensation  may  be  disturbed  in  individual  qualities  or 
all  of  its  qualities  may  become  involved.    According  to  our 


MENINGOMYELITIS  227 

present  experience  the  temperature  sense  occupies  a  pecu- 
liar position.  The  other  qualities  of  sensation  may  be  abso- 
lutely intact  and  temperature  sense  be  the  only  one  affected, 
and  vice  versa,  it  may  be  the  only  quality  of  sensation  re- 
maining intact  when  all  the  others  are  affected. 

The  dissociation  of  sensation  may  still  be  further  in- 
creased when  the  feeling  for  either  warm  or  cold  is  lost 
separately.  The  sense  of  pain  may  also  be  lost  alone  or 
both  the  temperature  sense  and  the  sense  of  pain  together 
may  be  affected,  the  other  qualities  remaining  preserved. 
In  general,  disturbance  of  sensation  is  not  usually  so  com- 
plete as  disturbance  in  motion. 

The  tendon-reflexes  are  usually  increased  and  Babinski's 
and  Oppenheim's  phenomena,  either  singly  or  together, 
present.  The  skin-reflexes  are  either  increased  or  diminished. 

A  weakness  of  the  bladder  function  belongs  to  the  early 
symptoms,  may  indeed  be  the  first  symptom  which  follows 
the  prodromal  symptoms  and  causes  the  patient  to  seek 
medical  advice.  It  is  apt  to  be  a  persistent  symptom  and  in 
those  cases  where  prompt  treatment  brings  about  a  disap- 
pearance of  the  motor  and  sensory  symptoms,  not  infre- 
quently the  bladder  weakness  remains  for  a  long  time  as  the 
only  remaining  trace  of  the  affection. 

Lumbar  Myelitis. — Myelitis  of  the  lumbar  cord  does  not 
occur  so  often.  Corresponding  to  our  knowledge  of  localiza- 
tion when  a  transverse  lesion  develops  in  the  lumbar  region 
a  flaccid  paralysis  occurs  and  both  the  tendon-  and  skin- 
reflexes  are  lost.  Concerning  the  behavior  of  sensation, 
what  has  just  been  said  in  regard  to  it  is  also  true  here. 
The  bladder  disturbance  may  be  in  the  form  of  a  detrusor 
or  sphincter  weakness,  or  a  combination  of  both.  The 
picture  of  ischuria  paradoxia  may  also  occur.  This  form  of 
myelitis  is  especially  prone  to  decubitus  and  severe  trophic 
disturbances.  The  tendon  and  periosteal  reflexes  of  the 
upper  extremity  without  any  disturbance  of  motion  or  sen- 
sation may  be  increased,  indicating  that  the  meninges  are 
hyperaemic. 

Cervical  Myelitis. — If  the  cervical  cord  is  affected  then 
all  four  extremities  undergo  motor  and  sensory  disturb- 


228  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

ances.  The  sensation  is  affected  particularly  here  in  the 
form  of  individual  qualities.  If  the  eighth  cervical  and 
first  dorsal  roots  are  involved  then  symptoms  of  an  affec- 
tion of  the  sympathetic  appear.  The  pupil  of  the  side  in- 
volved is  smaller,  the  bulb  is  sunken,  the  side  of  the  face 
shows  anomalies  in  the  sweat  secretion,  and  is  cooler  than 
normal.  These  symptoms  may  appear  singly  or  in  com- 
bination. 

Two  facts  worthy  of  mention  in  the  symptomatology 
should  be  spoken  of  here: 

1.  In   many   cases   brain   symptoms   precede   the   true 
spinal-cord   symptoms.     The  patients  suffer  from  severe 
headaches  or  attacks  of  dizziness  or  transient  a  phasic  dis- 
turbances or  unilateral  parsesthesias  or  paresis  in  the  ex- 
tremities. 

2.  In  all  forms  of  meningomyelitis  the  symptoms  may 
vary  in  extensiveness  and  intensity.    This  variation  in  the 
symptoms  is  particularly  emphasized  by  such  observers  as 
Westphal,  Oppenheim,  and  Siemerling  as  a  characteristic 
of  the  syphilitic  form  of  meningitis.    It  does  not  occur  in 
all  cases,  however,  and  such  an  experienced  observer  as 
Goldflam  has  never  been  able  to  find  the  change  in  the  patel- 
lar  reflexes  which  the  other  authorities  mention  so  prom- 
inently. 

The  following  case  is  an  example  of  the  most  frequent 
clinical  form  of  spinal  lues,  chronic  dorsal  and  cervico- 
dorsal  myelitis : 

The  case  is  one  of  a  woman,  thirty-eight  years  old,  whose 
husband  four  years  before  his  marriage  had  been  treated 
for  chancre  and  secondary  symptoms.  Six  weeks  before 
coming  under  my  observation  a  paresis  of  the  lower  ex- 
tremities with  a  weakness  of  the  bladder  and  girdle-pains 
in  the  region  of  the  nipples  had  developed.  Objectively  at 
the  time  of  the  examination  a  spastic  paresis  of  the  lower 
extremities  with  slight  diminution  of  sensation  for  all  quali- 
ties as  far  as  the  hips  could  be  demonstrated.  The  spinal 
column  was  intact.  The  motor  and  sensory  symptoms  pro- 
gressed to  such  an  extent  that  three  weeks  later  the  picture 
of  a  transverse  dorsal  myelitis  was  well  developed.  Under 


MENINGOMYELITIS  229 

antispecific  treatment  all  the  symptoms  gradually  disap- 
peared so  that  in  six  months'  time  the  patient  was  able  to 
walk,  although  tiring  easily  and  spastic  in  gait. 

The  following  case  is  interesting  for  two  reasons :  First, 
because  it  shows  a  spastic  paraparesis  of  the  lower  extremi- 
ties may  develop  in  other  ways  than  by  the  slow  progressive 
form.  Second,  that  energetic  antispecific  therapy  at  first 
did  not  influence  the  process  and  that  it  was  only  after  a 
long  pause  in  the  therapy  that  a  renewal  of  the  treatment 
succeeded  in  bringing  about  almost  a  complete  recovery. 

Specific  Cervical  Pachymeningitis,  Chronic  Dorsal  Myelitis. — 
An  unmarried  man,  thirty  years  old,  had  three  years  before 
his  present  illness  a.  chancre.  He  received  at  that  time  a 
course  of  inunctions  of  ten  weeks '  duration,  and  had  taken 
potassium  iodid  for  a  long  time.  Nine  months  ago  he  was 
seized  suddenly  with  a  paralysis  of  the  right  arm  with 
paragsthesias.  Under  a  three  months'  administration  of 
potassium  iodid  and  mercury  this  paralysis  almost  entirely 
disappeared.  Shortly  after  this  the  patient  complained 
of  lumbago-like  pains  in  the  back  which  lasted  eight  days. 
Three  months  ago  he  was  taken  with  a  suddenly-developing 
paralysis  of  the  left  leg,  which  in  the  course  of  two  weeks 
extended  to  the  right  leg. 

The  examination  revealed  a  slight  paresis  of  the  right 
arm,  a  typical  spastic  paresis  of  the  lower  extremities, 
with  slight  hypoaesthesia  for  all  qualities  up  to  the  nipples. 
Energetic  mixed  treatment  produced  at  first  no  improve- 
ment. Except  for  the  varying  intensity  of  the  spastic 
symptoms  the  condition  remained  practically  stationary  for 
nine  months.  Renewed  administration  of  the  mixed  treat- 
ment brought  about  a  gradual  improvement  so  that  at  the 
end  of  twelve  months  the  patient  was  able  to  walk  almost 
normally  without  a  cane.  Four  weeks  before  his  discharge 
from  the  hospital  there  remained  a  slight  spastic  paresis 
of  the  lower  extremities  with  a  slight  hypoaesthesia  for  all 
qualities  limited  to  the  legs.  The  left  pupil  was  larger  than 
the  right.  The  light  reaction  of  both  were  poor.  Oph- 
thalmoscopically,  there  was  a  grayish  appearance  of  the 
temporal  half  of  the  papillae. 


230  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

In  the  following  case  the  cardinal  symptoms  were  a  very 
considerable  degree  of  spastic  paraparesis  of  the  lower 
extremities  and  a  marked  interference  with  the  bladder 
function.  The  case  also  shows  the  incongruence  existing  in 
the  relationship  between  motion  and  sensation,  and  how 
marked  the  disturbance  of  one  quality  of  sensation,  in  this 
instance  the  muscle  sense,  may  be.  It  shows  further  the 
degree  of  variation  which  may  occur  in  the  motor  function 
and  how  futile  sometimes  a  thorough  and  prolonged  anti- 
specific  treatment  is  in  arresting  the  disease. 

The  patient,  a  man  thirty-one  years  old,  was  under  my 
care  in  the  hospital  fourteen  months.  He  had  always  been 
strong  and  had  a  good  family  history.  Two  and  one-half 
years  before  coming  under  my  care  he  had  contracted  syph- 
ilis. Eight  weeks  before  his  admission  into  the  hospital  he 
had  taken  a  course  of  inunctions.  At  the  examination  the 
internal  organs  appeared  normal  and  there  were  no  signs 
of  a  recent  or  past  syphilis.  Examination  of  the  nervous 
system  showed  a  spastic  paresis  of  the  lower  extremities. 
While  the  increase  of  the  tendon-reflexes  was  always  con- 
siderable, the  degree  of  muscle  tension  in  passive  motion 
varied  considerably ;  the  active  movements  of  the  lower  ex- 
tremities likewise  were  subjected  to  considerable  variation. 
The  gait  was  a  typical  spastic  one.  Sensation  in  the  region 
of  the  lower  limbs  was  slightly  affected  in  that  sense  of 
position  in  the  toes  and  the  ability  for  localization  on  the 
feet  and  legs  was  disturbed;  otherwise  sensation  was  nor- 
mal. Ataxia  and  Eomberg  were  not  present.  No  other 
anomalies  of  the  body  or  upper  extremities  were  observed. 
During  the  following  six  months  the  patient  was  given  a 
combination  treatment  of  mercury  and  potassium  iodid. 
He  complained  often  of  lancinating  pains  radiating  from 
the  back  and  loins  down  into  the  thighs.  The  bladder  weak- 
ness which  expressed  itself  in  frequent  urination  and 
enuresis  improved  slightly.  The  spastic  paresis  with  slight 
remissions  gradually  increased.  The  disturbance  of  sensa- 
tion remained  unchanged.  Six  months  later  the  sensation 
in  the  lower  extremities  had  grown  worse  in  that  all  the 


MENINGOMYELITIS  231 

qualities  were  affected.  The  spastic  symptoms  were  severe. 
The  patient  was  only  able  to  drag  himself  around  in  his 
room  by  the  aid  of  two  canes  with  difficulty.  When  he 
attempted  to  move  his  limbs  in  bed  involuntary  muscle 
spasms  appeared  which  produced  a  marked  tremor  in  the 
legs.  Thorough  and  continued  treatment  made  practically 
no  change  in  the  patient's  condition  and  he  was  finally  dis- 
charged from  the  hospital. 

In  this  case  we  may  assume  that  it  was  one  of  meningo- 
myelitis  which  was  localized  in  the  lower  dorsal  and  lumbar 
regions.  The  radiating  pains  indicated  an  involvement  of 
the  meninges.  The  secondary  involvement  of  the  medullary 
fibres  had  caused  an  irreparable  injury  to  them  with  a 
descending  secondary  degeneration.  Whether  a  primary 
arterial  disease  with  the  consequent  thrombotic  softening 
of  the  medullary  substance  existed  was  not  able  to  be  defin- 
itely determined. 

The  Course  of  Meningomyelitis. — The  course  of  meningo- 
myelitis  is  a  varied  one.  Usually  the  disease  begins  chroni- 
cally and  continues  chronic  in  character,  yet  now  and  then 
it  sets  in  acutely  with  a  motor  paresis  of  one  or  more 
extremities.  Generally  the  course  is  uninterrupted  and 
progressive,  but  not  infrequently  it  may  become  quiescent 
and  remain  so  for  months  and  even  years.  Intermittent  im- 
provement also  sometimes  occurs. 

In  addition  to  the  motor  improvement,  sensation  and 
the  disturbance  of  the  bladder  functions  may  improve  won- 
derfully. In  some  cases  the  improvement  is  only  a  tem- 
porary one  and  a  relapse  occurs ;  in  others  it  is  permanent ; 
in  still  others  contractions  develop  which  follow  as  a  conse- 
quence of  the  myelitic  lesion  and  the  secondary  degenera- 
tions arising  from  it. 

There  are  a  small  number  of  cases  which  grow  progres- 
sively worse  and  exhibit  a  complete  paralysis,  both  of 
sensation  and  motion.  In  these  cases  death  is  usually 
caused  by  the  severe  decubitus  and  the  injurious  effects 
of  the  severe  bladder  paralysis  on  the  bladder  and  kidneys. 

At  any  stage  the  chronic  paraparesis  may  be  trans- 
formed by  an  acute  attack  into  a  complete  transverse 


232  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

paralysis,  or  in  other  words,  the  chronic  myelitis  may  sud- 
denly be  changed  into  a  transverse  acute  myelitis. 

Erb's  Syphilitic  Spinal  Paralysis. — The  most  common  form 
of  spinal-cord  syphilis  is  that  of  complete  transverse  dorsal 
myelitis  which  Erb,  in  1892,  described  in  the  following 
manner : 

Erb's  Definition. — "Several  years  after  a  specific  infec- 
tion, the  slow  and  gradual  development  of  a  spastic  paresis 
of  the  lower  extremities  with  spastic  gait,  motor  paralysis, 
slight  muscle  tension,  slight  increase  of  the  tendon-reflexes, 
sensory  disturbances  either  slight,  severe,  or  absent,  and 
bladder  weakness.  The  upper  extremities  as  well  as  the 
intelligence,  the  cranial  nerves,  and  the  pupils  remaining 
normal." 

Such  observers  as  Kuh,  Gerhardt,  Mendel,  and  Lamy 
have  acknowledged  Erb's  clinical  description  as  character- 
istic of  spinal  syphilis,  but  have  also  pointed  out  the  fact 
that  this  symptom-complex  is  found  in  other  affections  of 
the  cord.  Oppenheim,  Leyden,  and  Goldschneider  have  ex- 
pressed the  view  that  Erb's  symptom-complex  is  only  a 
stage  in  syphilitic  meningomyelitis.  From  Erb's  original 
work  it  is  evident  that  he  allows  to  the  borders  of  specific 
spinal  paralysis  a  fairly  wide  latitude.  On  the  one  hand 
we  see  the  almost  pure  type  of  spastic  spinal  paresis,  a 
slowly  developing  spastic  paresis  of  the  lower  extremities 
with  slight  contractures,  entirely  lacking  or  only  slightly 
indicated  sensory  disturbances,  almost  entirely  normal 
bladder  and  sexual  functions;  on  the  other  hand  we  see 
subacutely  appearing  motor  paraplegias  with  muscle  con- 
tractures and  increased  tendon-reflexes,  rather  marked  ob- 
jective sensory  disturbances  with  impotence,  severe  sphinc- 
ter disturbances,  and  myelitic  decubitus. 

The  time  of  the  beginning  of  the  disease  after  the  infec- 
tion likewise  comes  within  wide  borders,  one  and  one-half 
to  twenty-four  years. 

The  pathology  of  Erb's  symptom-complex  has  been  for- 
mulated by  him  and  his  pupil  Sidney  Kuh,  without  the  cor- 
roboration  of  an  autopsy,  Jiowever,  as  follows:  A  partial 
transverse  lesion  is  assumed  and  a  symmetrically  situated 


MENINGOMYELITIS  233 

affection  of  both  halves  of  the  cord.  The  root  regions  of 
Burdach's  columns  are  not  involved;  the  posterior  parts  of 
these  columns,  together  with  the  lateral  and  pyramidal 
tracts,  are  affected;  the  posterior  gray  columns  and  the 
posterior  white  tracts  are  also  involved;  the  anterior  half 
of  the  cord  remains  intact;  perhaps  the  cerebellar  tracts 
and  the  columns  of  Goll  are  affected. 

The  degeneration  depends  upon  a  syphilitic  infiltration 
of  the  medullary  substance,  or  a  luetic  change  which  origi- 
nates from  luetic  disease  of  the  arteries.  Through  this 
arterial  disease  Erb  explains  the  sometimes  rapid,  some- 
times gradual  beginning  of  the  affection,  also  the  remissions 
in  the  course  of  the  disease  and  the  usually  only  partial 
success  of  the  therapy. 

The  position  of  Kuh  in  his  comprehensive  work  on  this 
subject  is  against  the  assumption  of  a  primary  sclerosis. 
His  argument  is  that  a  primary  sclerosis  in  the  spinal  cord 
appears  only  a  long  time  after  the  infection  and  would  not 
be  influenced  by  antispecific  therapy.  It  is  certainly  true, 
as  Kuh  remarks,  that  the  cases  with  an  acute  beginning 
and  the  cases  where  myelitis  develops  early  are  not  due  to  a 
sclerosis,  but  it  must  also  be  admitted  that  this  objection 
does  not  apply  to  the  great  majority  of  cases  of  "syphilitic 
spinal  paralysis"  which  develop  gradually,  and  slowly,  and 
that  further,  in  many  cases  the  treatment  does  not  produce 
any  marked  remission  and  that  such  remissions  may  be 
observed  spontaneously  in  sclerosis. 

At  the  present  time  the  pathological  examinations  of  the 
cases  of  "syphilitic  spinal  paralysis"  which  have  come  to 
autopsy  have  pretty  definitely  settled  that  the  pathology 
of  the  uncomplicated  forms  of  Erb's  paralysis  is  a  primary 
combined  system  disease  without  any  particular  disease  of 
the  arteries  and  without  meningitis.  It  has  also  been  deter- 
mined that  the  clinical  picture  of  spastic  spinal  paralysis 
with  slight  weakness  of  the  bladder  and  trifling  involvement 
of  sensation  occurs  both  in  syphilitics  and  non-syphilitics, 
and  in  both  the  pathological  basis  for  the  symptoms  is  due 
to  a  combined  system  disease  in  the  lateral  columns  (cross- 
pyramidal  tracts)  and  the  columns  of  Goll. 


234  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

The  symptoms  of  syphilitic  spinal  paralysis  may  ap- 
pear early.  In  the  22  cases  reported  by  Erb,  13  began 
within  the  first  three  years  after  the  infection.  In  three  of 
my  own  cases,  one  developed  in  the  twenty-fourth  year,  one 
in  the  sixth,  and  one  a  year  and  a  half  after  the  infection. 
In  the  light  of  our  present  knowledge,  both  clinical  and 
pathological,  one  is  able  to  form  some  definite  conclusion 
with  reference  to  " syphilitic  spinal  paralysis." 

1.  There    are   without   doubt    cases    that   present   the 
symptom-complex  which  Erb  has  described  for  many  years 
until  death.     There  are  also  cases  in  which  the  symptom- 
complex  represents  not  only  a  "syndrome"  but  the  disease 
itself. 

2.  The  pathological  basis  of  this  affection  is  due  either 
to  primary  combined  tract  affection  in  the  posterior  and 
lateral  columns  without   specific  disease  of  the  cord,  in 
which  case  it  can  be  regarded  either  as  a  true  system  affec- 
tion or  tract  disease  arising  from  a  chronic  myelitis,  which- 
ever one  may  choose  to  regard  it,  or  its  pathological  basis 
consists  of  this  same  tract  disease  in  combination  with  a 
diffuse  chronic  myelitic  process  in  which  the  spinal-cord 
arteries  are  involved  with  an  arteritis  of  Heubner's  type, 
the  meninges  are  affected  to  a  greater  or  less  degree. 

According  to  our  present  experience  the  latter  form  is 
the  most  frequent. 

3.  It  is  further  true,  that  in  many  cases  Erb's  symptom- 
complex  forms  only  the  basis  of  the  clinical  picture,  which 
finally  ends  either  in  spastic  contractures  or  incomplete 
transverse  myelitis.     In  such  cases  the  symptom-complex 
represents  only  a  syndrome.    In  these  cases  we  have  patho- 
logically on  the  one  hand  a  pure  type  of  sclerosis  of  the 
lateral  columns  in  which  arterial  changes  of  the  specific 
variety  do  not  belong  and  in  which  a  more  or  less  extensive 
chronic  myelitis  may  occur,  and  on  the  other,  either  a 
primary  combined  tract  disease  in  conjunction  with  a  more 
or  less  extensive  myelitis  only,  with  ascending  and  descend- 
ing degenerations. 

Finally  we  are  confronted  with  the  question  as  to 
whether  this  symptom-complex  is  so  characteristic  that  in 


MENINGOMYELITIS  235 

all  probability  its  presence  means  the  existence  of  a  syphilo- 
genetic  process  in  the  spinal  cord.  Erb  answers  this  ques- 
tion in  the  affirmative.  Leyden  and  Groldschneider  on  the 
other  hand  do  not  regard  it  as  pathognomonic  of  lues  and 
claim  it  may  be  observed  in  diffuse  non-specific  affections 
of  the  cord. 

According  to  my  own  experience  I  do  not  believe  that  the 
typical  form  occurs  except  from  a  specific  origin,  but  I  do 
believe  that  as  soon  as  the  bladder  symptoms  and  dis- 
turbances of  sensation  become  more  severe  and  muscle  rigid- 
ity is  added  to  the  clinical  picture,  the  differential  diag- 
nosis from  multiple  sclerosis,  extramedullary  tumor,  and 
pseudo-system  disease  such  as  is  found  in  anaemias  becomes 
extraordinarily  difficult. 


XII 

SYPHILIS  OF  THE  SPINAL  CORD 

Acute  Specific  Transverse  Myelitis. — Syphilitic  spinal-cord 
paralysis  may  run  an  acute  course  from  its  very  beginning, 
and  the  chronic  type  of  specific  myelitis  may  also  develop 
into  an  acute  transverse  myelitis  at  any  time.  The  acute 
transverse  myelitis  is  not  an  infrequent  form  of  spinal 
syphilis. 

Frequency. — Orlowsky,  in  72  cases  of  spinal  syphilis, 
found  the  acute  type  in  19  cases.  In  92  cases  in  my  private 
practice  and  120  hospital  cases  I  have  observed  in  the 
former  3  cases  and  in  the  latter  5  cases  of  acute  myelitis. 

Mode  of  Appearance. — The  transverse  paralysis  either  de- 
velops very  quickly,  in  a  few  hours  the  lower  extremities 
becoming  paraplegic,  or  it  requires  several  days  for  its  com- 
plete development.  The  paralysis  is  almost  always  an  abso- 
lute one,  so  that  from  the  pelvis  down  all  voluntary  motion 
is  gone.  According  to  the  level  of  the  lesion  in  the  cord  the 
lower  extremities  alone  are  involved,  or  in  a  higher  lesion 
a  portion  of  the  body  as  well.  The  upper  extremities  are 
rarely  affected. 

Sensation. — Sensation  is  usually  severely  affected.  Either 
all  the  qualities  of  sensation  are  gone  or  individual  quali- 
ties, especially  the  temperature  sense  over  a  varying  degree 
of  surface,  may  be  preserved;  also  the  sense  of  touch  may 
be  retained.  Thermo-analgesia  may  sometimes  represent 
the  only  sensory  disturbance. 

Acute  specific  myelitis  may  present  the  Brown-Sequard 
complex  for  longer  or  shorter  periods  in  the  course  of  the 
disease. 

The  tendon-reflexes  are  abolished  when  the  lesion  is 
located  in  the  lumbar  cord,  or  preserved  or  increased  when 
situated  above  the  centres  for  these  reflexes.  The  sphinc- 
ters of  the  bladder  and  rectum  are  from  the  beginning 
severely  involved.  In. many  cases  osdema  quickly  makes  its 

236 


SYPHILIS  OF  THE  SPINAL  CORD  237 

appearance  in  the  paralyzed  parts  and  decubitus  developing 
in  the  sacral  region  and  rapidly  progressing  is  a  frequent 
occurrence. 

In  a  few  cases  the  paralysis  appears  when  the  patient 
is  apparently  in  complete  health.  In  the  majority  of  cases, 
however,  premonitory  symptoms  precede  the  appearance 
of  the  paralysis.  The  paralysis  usually  appears  in  an  in- 
dividual who  although  limited  in  his  ability  to  work  is  still 
able  to  go  around. 

Prodromal  Symptoms. — Rosin  divides  the  prodromal 
symptoms  into  sensory  and  motor  symptoms  and  dis- 
turbances of  the  functions  of  the  bladder  and  rectum.  The 
sensory  symptoms  consist  in  paraesthesias  and  radiating, 
shooting,  and  boring  pains  in  the  lower  extremities,  in  the 
hips  and  in  certain  parts  of  the  trunk  and  spinal  column, 
also  a  certain  stiffness  of  the  back.  Sensitiveness  to  move- 
ments of  the  trunk  may  be  mentioned  here.  These  are  the 
symptoms  of  spinal  irritation  upon  which,  many  years  ago, 
Charcot  laid  great  weight,  and  more  recently  Oppenheim 
has  again  pointed  out  as  of  frequent  occurrence  and  rela- 
tively characteristic. 

The  motor  prodromal  symptoms  manifest  themselves  not 
only  as  irritative  symptoms  but  also  as  symptoms  of 
paralysis  as  well.  Twitchings  and  spasm-like  movements  of 
the  toes,  feet,  and  legs  alternate  with  weakness  and  with 
actual  paretic  conditions. 

Very  often,  as  Williamson  especially  has  called  atten- 
tion to,  disturbance  of  the  bladder  and  rectum  in  the  form 
of  retention  precedes  the  appearance  of  the  transverse 
paralysis. 

Rosin  claims,  after  a  review  of  the  literature  on  this 
subject,  that  a  prodromal  stage  may  often  be  recognized 
from  the  behavior  of  the  patellar  reflexes,  which  change  at 
different  times  from  being  increased,  then  to  normal  and 
diminished.  These  prodromal  symptoms  last  in  many 
cases  not  only  days  and  weeks,  but  months,  and  even  years. 
Their  specific  nature  may  be  indicated  to  the  careful  obser- 
ver by  their  fleeting  character  and  tendency  to  recover. 

The  Course. — In  the  well-developed  form  of  the  disease, 


238  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

relatively  frequent  in  comparison  to  the  non-specific  type 
of  transverse  myelitis,  a  variation  in  the  degree  of  paralysis 
of  both  sensation  and  motion  occurs.  An  improvement  may 
take  place  entirely  independent  of  the  therapy  which  lasts 
for  days  and  weeks  and  then  disappears.  The  disease 
usually  runs  a  rather  rapid  and  fatal  course. 

iSchmaus  reports  a  case  which  terminated  in  death  after 
fourteen  days,  Williamson  one  after  fifteen  days,  Groldflam 
two  cases  which  ran  courses  of  four  and  six  weeks,  respec- 
tively. 

Case  of  acute  specific  myelitis  of  the  lumbar  cord:  A 
woman,  forty-six  years  old,  had  contracted  syphilis  ten 
years  previously  and  had  received  treatment  for  it  at  two 
different  periods. 

Two  years  before  her  present  illness,  after  several  weeks 
of  prodromal  symptoms  in  the  form  of  girdle-pains  and 
pains  radiating  down  the  limbs,  she  became  suddenly 
paralyzed  in  the  lower  extremities.  This  paralysis  cleared 
up  in  the  course  of  a  few  weeks  under  the  administration 
of  potassium  iodid,  with  the  exception  of  some  weakness 
which  still  remained  in  the  limbs.  The  patient  was  able 
to  resume  her  work  for  a  space  of  two  years,  until  one  day, 
without  any  external  cause,  with  sharp  pains  in  the  limbs 
and  back,  she  again  became  paralyzed.  This  time  there 
was  a  complete  motor  and  sensory  paralysis,  with  loss  of 
the  reflexes  and  paralysis  of  the  bladder  and  rectum.  Decu- 
bitus  quickly  appeared  and  rapidly  progressed,  and  the 
patient  died,  with  symptoms  of  sepsis,  in  two  months '  time. 
At  the  autopsy  as  stigma  of  an  earlier  lues  considerable 
atheromatous  degeneration  of  the  beginning  aorta,  the  pic- 
ture of  Heller's  aortitis  was  found.  The  brain  was  normal. 
In  the  lumbar  part  of  the  spinal  cord  the  dura  and  pia  were 
adherent.  The  cord-substance  was  soft,  almost  pulpy. 

The  microscopical  examination  showed  the  anterior  and 
posterior  spinal  arteries,  as  well  as  all  the  arteries  in  the 
anterior,  and  some  in  the  posterior  roots  affected  with  a 
severe  arteritis  of  the  Heubner  type.  In  many  of  the  ves- 
sels the  size  of  the  lumen  was  reduced  to  a  minimum.  The 
vessels  in  a  transverse  section  of  the  lumbar  cord  were 


SYPHILIS  OF  THE  SPINAL  CORD 


239 


markedly  increased.  The  walls  of  all  were  thickened  and 
the  lumina  narrowed  and  in  some  instances  obliterated 
entirely.  Above  the  lesion  there  was  recent  secondary  dis- 
ease of  the  columns  of  Goll. 

Genuine  inflammatory  symptoms  were  absent,  also  gum- 
matous  disease.  The  pia  mater  was  more  or  less  strongly 
proliferated  and  thickened.  The 
disease  of  the  vessels  was  apparent 
in  all  the  different  levels  of  the 
cord  where  transverse  sections 
were  made,  but  most  marked  in 
the  lumbar  region. 

The  proliferation  of  the  pia  the 
higher  up  one  got  gradually  grew 
less,  until  finally  in  the  cervical 
region  it  was  not  to  be  observed. 

This  case  presents  a  typical  ex- 
ample of  acute  specific  myelitis. 
Two  years  before,  the  process  had 
given  serious  warning  in  the  pro- 
dromal symptoms  above  described. 
Then  suddenly  a  transverse  soft- 
ening appeared  as  a  result  of  dis- 
turbances of  nutrition  caused  by 
severe  disease  of  the  vessels.  This 
process  was  irreparable. 

In  the  majority  of  cases  cystitis 
and  decubitus  in  those  completely 
paralyzed  cause  death  in  a  few 
months. 

In  rare  cases,  by  careful  atten- 
tion, life  may  be  preserved  for  a 
long  time.  In  one  of  my  cases, 
which  was  completely  paralyzed 
from  the  navel  down,  in  spite  of  a  total  paralysis 


Fios.  56-59. 


of 


both  bladder  and  rectum,  it  was  possible,  by  scrupulous 
care,  to  keep  the  cystitis  and  decubitus  within  reasonable 
limits  and  to  prolong  life  for  ten  years.  The  patient  finally 
died  of  laryngeal  carcinoma. 


240  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

Sometimes  marked  and  lasting  improvement  is  observed, 
as  in  the  following  case : 

A  woman,  forty  years  old,  with  a  previous  history  of 
lues,  had,  three  months  before  I  saw  her,  without  any  pre- 
monitory symptoms  or  any  demonstrable  cause,  developed 
a  severe  motor  paralysis  of  the  lower  extremities,  with  total 
paralysis  of  the  bladder  and  rectum.  Decubitus  on  the  hips 
appeared.  Cerebral  symptoms  were  absent.  Objective  sen- 
sory disturbances  were  absent.  Energetic  mixed  treatment 
was  administered,  and  with  such  a  degree  of  success  that 
after  three  months  the  patient  was  able  to  walk  with  the 
aid  of  a  cane.  Her  condition  at  the  present  time,  after 
three  years,  remains  about  the  same,  namely,  typical  spastic 
paresis  of  the  lower  extremities,  with  weakness  of  the  blad- 
der and  rectum. 

In  this  case  premonitory  symptoms  were  entirely  absent, 
likewise  the  incongruence  between  the  motor  and  sensory 
paralysis  with  sphincter  involvement  was  marked. 

Differential  Diagnosis,  Tuberculosis. — In  the  differential 
diagnosis  those  cases  in  which  some  other  infection  or  in- 
toxication can  be  demonstrated  are  to  be  excluded.  Not 
infrequently  differential  diagnostive  difficulties  occur  in  the 
form  of  an  acute  transverse  myelitis  that  takes  place  as  a 
result  of  spinal  caries  and  the  tubercular  exudate  produced 
by  it.  Prodromal  symptoms  here  also  are  usually  present 
for  a  longer  or  shorter  period.  The  characteristic  gibbus 
formation  may  not  always  be  present.  In  such  cases  the 
Rontgen  ray  is  of  decided  advantage.  Tuberculosis  of  the 
spinal  column,  however,  usually  exhibits  the  clinical  picture 
of  a  gradual  compression,  and  almost  always — exceptions 
do  sometimes  occur— elsewhere  in  the  body  tuberculosis  can 
be  demonstrated.  The  history  of  a  previous  syphilitic  in- 
fection does  not  exclude  tuberculosis.  In  general,  it  may 
be  said  that  tuberculosis  can  be  excluded  in  an  acute  trans- 
verse myelitis  when  in  a  patient  who  previously  has  had 
syphilis  neither  symptoms  of  tuberculosis  can  in  other  por- 
tions of  the  body  be  found  nor  caries  of  the  spine 
demonstrated. 


SYPHILIS  OF  THE  SPINAL  CORD  241 

Malignant  Tumor. — The  differential  diagnosis  is  still 
more  difficult  in  those  rare  cases  of  malignant  tumor  of  the 
vertebral  column  in  which  the  tumor  extends  from  the  spine 
to  the  meninges,  surrounds  the  cord,  and  leads  to  a  trans- 
verse softening.  If  there  is  a  previous  history  of  syphilis 
in  such  cases  a  wrong  diagnosis  is  almost  inevitable. 

Spinal  Apoplexy. — Finally  one  must  take  into  considera- 
tion those  rare  cases  of  spinal  apoplexy  in  which  entirely 
acute  with  prodromal  symptoms  of  only  a  few  hours'  dura- 
tion a  complete  transverse  paralysis  occurs  in  a  previously 
entirely  healthy  person  and  without  any  demonstrable 
cause.  The  following  is  such  a  case: 

The  wife  of  a  school  teacher,  thirty  years  old,  was  sud- 
denly one  night  seized  with  pains  in  the  back  which  radiated 
down  into  the  limbs.  The  next  morning  she  could  only  with 
difficulty  move  her  limbs,  and  by  noon  on  the  same  day 
was  completely  paraplegic  in  both  the  motor  and  sensory 
functions,  including  a  paralysis  of  the  sphincters.  This 
condition  has  existed  for  six  years  without  any  variation. 
One  must  assume  in  such  cases  that  a  spinal-cord  hemor- 
rhage exists,  the  cause  of  which  is  not  clear,  analogous  to 
the  hemorrhage  which  sometimes  occurs  in  the  brain  of 
young  and  healthy  persons. 

Pathology. — The  pathology  of  acute  paraplegia  in  syph- 
ilis may  embrace  the  meninges  and  spinal  cord  together, 
or  either  one  alone.  In  the  majority  of  cases  the  patho- 
logical findings  indicate  syphilis.  Occasionally  cases  are 
encountered  where  there  is  nothing  to  distinguish  between 
specific  and  non-specific  myelitis.  One  finds  in  the  meninges 
either  only  disease  of  the  blood-vessels,  such  as  is  usually 
found  in  specific  affections,  or  accompanying  this  also  in- 
flammatory infiltrations  around  the  vessels,  or  in  addition 
to  a  well-marked  meningitis  both  pachy-  and  lepto-,  some- 
times gummatous  processes. 

The  spinal  cord  itself  shows  a  greater  or  less  degree  of 
arterial  disease. 

Irregular  foci  of  softening,  or  sclerosis,  or  a  large  trans- 
verse lesion  which  is  usually  situated  in  the  mid-dorsal 
region,  or  a  necrosis  of  the  gray  matter  along  with  changes 

16 


242  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

in  the  white  matter,  or  the  penetration  of  gummatous 
masses  from  the  meninges  into  the  white  substance,  or, 
finally,  the  chief  pathological  lesion  may  consist  of  a  hemor- 
rhage in  the  gray  matter  with  secondary  necrosis  of  the 
same.  At  the  same  time  one  finds  in  the  arteries  endo-, 
peri-,  and  mesoarteritis.  Sottas  found  in  the  walls  of  the 
arteries  even;  small  gummata.  Thrombosis  of  both  the 
larger  and  smaller  spinal  arteries,  and  also  thrombosis  of 
the  endo-  and  periphlebitic  affected  veins  is  of  common 
occurrence.  The  foci  of  softening  are  found  most  fre- 
quently in  the  periphery  of  the  lateral  and  posterior  col- 
umns and  next  in  frequency  in  the  anterior  gray  matter. 

In  the  majority  of  cases  the  pathology  consists  of  a 
combination  of  the  different  above-mentioned  changes,  a 
varied  admixture  of  vessel  disease  and  its  consequences, 
inflammatory  and  less  frequently  specific  gummatous  infil- 
trations. In  addition  to  these  processes  there  is  also  an 
ascending  and  descending  degeneration. 

The  pathology  explains  in  the  best  way  the  clinical 
picture,  and  especially  it  explains  those  features  which 
strike  one  as  differing  to  a  greater  of  less  degree  from 
the  ordinary  acute  myelitis. 

The  prodromal  symptoms,  the  spinal  rigidity,  the  pains 
in  the  back,  the  sensory  and  motor  symptoms  of  irritation 
in  the  extremities,  which  vary  in  intensity  and  localization, 
are  best  explained  by  the  slow  and  intermittent  develop- 
ment of  the  infiltration  and  inflammation  of  the  meninges. 
The  fact  that  the  spinal  cord  remains  for  so  long  a  time 
apparently  intact  is  partly  for  the  reason  that  the  forma- 
tion of  thrombosis  and  the  production  of  impermeability  in 
the  blood-vessels  requires  time,  even  though  they  may  be 
severely  affected,  and  partly  because  vessels  which  are  not 
involved  or  only  slightly  involved  may  act  as  compensatory. 

The  Incongruence  Between  the  Pathology  and  the  Clinical 
Symptoms. — The  relative  frequent  incongruity  between  the 
motor  and  sensory  paralysis  may  be  explained  by  the  in- 
volvement or  non-involvement  of  the  arteries  which  supply 
the  motor  and  sensory  areas  of  the  cord.  The  circumstance 
that  the  localization  of  the  final  paralysis  does  not  always 


SYPHILIS  OF  THE  SPINAL  CORD  243 

correspond  to  that  of  the  prodromal  symptoms  depends 
on  the  fact  that  the  meningitic  process  is  independent  of 
the  thrombotic  formation  in  the  vessels. 

Simple  Acute  Myelitis  in  Syphilitics. — Cases  of  acute  trans- 
verse myelitis  have  occasionally  been  reported  in  syph- 
ilitics  as  occurring  shortly  after  the  infection  in  the  early 
secondary  stage,  which  pathologically  cannot  be  differen- 
tiated from  the  ordinary  myelitis  of  softening.  The  fol- 
lowing case  is  of  this  form: 

A  seaman,  thirty-nine  years  old,  two  years  after  a  speci- 
fic infection  and  four  weeks  after  a  slight  injury  to  his  back, 
became  acutely  paraplegic.  He  was  bed-ridden  in  my  de- 
partment at  Oppendorf  for  eight  years  with  all  the  symp- 
toms of  a  complete  transverse  lesion  in  the  lower  dorsal 
region,  and  finally  died  of  a  pneumonia.  The  microscopical 
examination  of  this  case  showed  a  simple  degeneration  of 
the  entire  transvere  cord  from  the  eighth  to  the  eleventh 
dorsal  segments.  There  were  no  specific  changes  either 
in  the  cord  or  membranes  and  only  a  simple  thickening 
of  the  walls  of  the  vessels. 

Savard  called  attention  in  1882  to  the  not  infrequent 
occurrence  of  a  simple  acute  myelitis  in  persons  with  a 
specific  history  in  which  other  etiological  factors  could  not 
be  demonstrated.  Oppenheim,  in  his  well-known  work  con- 
cerning acute  myelitis,  has  also  attributed  to  syphilis  an 
etiological  role  which  causes  a  different  pathology  than  that 
of  the  specific  changes  which  have  been  described.  This 
pathology  is  produced  primarily  by  the  toxin  of  the  disease. 
These  cases  are  not  cases  of  spinal-cord  syphilis  but  cases 
of  acute  myelitis  depending  upon  a  syphilitic  basis. 

In  this  connection  the  following  case  is  of  interest : 

A  lawyer,  forty-five  years  old,  had  contracted  syphilis 
twenty  years  before  and  had  been  thoroughly  treated  for  it. 
The  patient  had  been  married  fifteen  years  and  had  four 
healthy  children.  He  was  taken  sick  with  paraesthesias  in 
the  lower  extremities  and  slight  dysuria.  Because  the 
pupils  did  not  react  promptly  and  the  tendon-reflexes  were 
difficult  to  obtain,  I  diagnosed  the  case  as  one  of  beginning 
tabes  and  sent  the  patient  to  Olynhausen.  Here,  while 


244  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

undergoing  a  course  of  inunctions,  severe  pains  appeared 
in  the  neck,  which  were  accompanied  by  a  rapidly  increas- 
ing motor  weakness  of  the  lower  extremities.  The  patient 
was  taken  home  and  the  picture  of  a  rapidly  developing 
complete  transverse  dorsolumbar  myelitis  was  presented. 
Paralysis  of  the  bladder,  with  the  resulting  cystopyelitis, 
and  some  decubitus  were  also  present. 

As  the  pains  in  the  neck  did  not  yield,  and  also  a  certain 
amount  of  stiffness  in  the  neck  existed  on  passive  motion, 
and  as  energetic  antispecific  treatment  proved  of  no  avail, 
the  probability  of  a  rapidly  growing  extramedullary  tumor 
was  considered  and  the  patient  subjected  to  an  exploratory 
laminectomy  with  a  negative  result.  Death  followed  soon 
after  the  operation. 

The  diagnosis  before  the  autopsy  was  made  of  tabes  dor- 
salis  and  meningomyelitic  lues. 

The  autopsy  revealed  in  the  middle  and  lower  cervical 
and  upper  dorsal  a  rather  marked  leptomeningitis,  which 
microscopically  was  still  of  an  acute  inflammatory  char- 
acter, but  did  not  show  any  gummatous  processes.  On 
transverse  section  of  the  cord  incipient  tabes  was  found  to 
be  present.  Otherwise,  with  the  Weigert  and  borax-carmine 
stains,  no  other  anomalies  were  found.  There  was  neither 
syphilitic  nor  other  disease  of  the  vessels  worth  mentioning, 
nor  an  acute  or  subacute  change  in  the  nervous  parenchyma. 

In  this  exceedingly  remarkable  case  one  is  also  forced 
to  the  assumption  that  a  severe  toxin  caused  the  serious 
disturbance  of  function  in  the  cord.  Figs.  60-64  are  from 
preparations  taken  from  this  case. 

It  is  not  altogether  improbable  that  preparations  stained 
according  to  the  Marchi  method  might  have  disclosed  alter- 
ations in  the  parenchyma.  At  any  rate  the  case  is  instruc- 
tive in  showing  the  striking  incongruity  which  can  exist 
between  the  clinical  symptoms  and  the  pathology. 

Landry's  Paralysis. — It  is  still  a  matter  of  controversy 
whether  in  individual  cases  syphilis  may  be  responsible  for 
the  clinical  syndrome  of  Landry's  paralysis.  Numerous 
cases  have  been  reported  by  different  observers  which  would 
seem  to  indicate  that  it  may  be.  Heubner,  from  his  own 


SYPHILIS  OF  THE  SPINAL  CORD 


245 


experience  and  also  the 
observations  of  others, 
assumes  that  there  is  a 
syphilitic  form  of  Lan- 
dry's  paralysis  which  ap- 
pears in  the  early  stages 
of  syphilis  without  either 
prodromal  or  meningitic 
symptoms.  It  may  be  con- 
s  i  d  e  r  e  d  the  exception 
rather  than  the  rule  that 
cases  of  acute  ascending 
spinal  paralysis  without 
any  pathology  have  any 
connection  with  syphilis. 

Disease  of  the  Anterior 
Gray  Substance. — The  an- 
t  e  r  i  o  r  gray  matter  is 
frequently  affected,  but 
usually  only  in  conjunc- 
tion with  a  myelitis  which 
extends  over  the  cord 
transversely.  The  i  n  - 
volvement  of  this  part  of 
the  cord  is  manifested  by 
atrophic  paralysis  with 
quantitative  and  qualita- 
tive changes  in  the  elec- 
trical responses. 

The  pathology  is  that 
of  a  simple  shrinking  and 
atrophy  of  the  ganglion- 
cells  and  their  axis-cylin- 
der processes,  as  well  as 
a  necrosis  of  the  network 
of  the  medullated  fibres; 
occasionally  a  deposit  of 
round  cells  is  found.  Ja- 
risch  believes  in  the  cases 


FIGS.  60-64. 


246 


SYPHILIS  AND  THE  NERVOUS  SYSTEM 


of  children  with  evident  syphilis,  where  he  has  had  an 
opportunity  to  examine  the  spinal  cord,  that  he  has  found 
the  ganglion-cells  in  the  gray  matter  reduced  in  size  and 
number  and  the  protoplasm  changed.  Whether  this  find- 
ing can  be  regarded  as  pathological,  and  whether  there  is 
any  relationship  between  it  and  florid  syphilis,  is  uncertain. 


Fio.  65. — Poliomyelitis  anterior  chronica,  choroiditis  luetica. 

Cases  of  poliomyelitis  subacuta  have  been  reported  in 
syphilitics,  but  in  such  cases  as  came  to  autopsy  the  patho- 
logical picture  did  not  differ  from  the  usual  atrophy  of  the 
cells  in  the  anterior  horns  and  exhibited  nothing  character- 
istic of  syphilis. 

I  have  observed  three  cases,  one  in  a  woman  and  two 


SYPHILIS  OF  THE  SPINAL  CORD  247 

in  men,  who  were  luetics,  in  which  the  clinical  symptoms 
presented  were  those  of  a  chronic  progressive  anterior 
poliomyelitis.  In  all  three  cases  only  the  upper  extremities 
were  affected,  in  two  cases  active  symptoms  of  syphilis  were 
present,  and  in  all  three  cases,  after  a  protracted  anti- 
specific  therapy,  the  disease  became  quiescent. 

In  the  woman  the  disease  ceased  to  progress  after  the 


Fio.  66. — Poliomyelitis  anterior  chronica   (lues  previously).     The  upper  extremities  are  com- 
pletely paralyzed  and  atrophic. 

muscles  of  the  hands,  the  forearms,  and  arms  had  become 
completely  paralyzed  and  atrophic  (Fig.  65).  In  one  of 
the  men  the  atrophic  paralysis  involved  the  shoulder-girdle 
and  a  portion  of  the  arm  and  forearm  musculature.  In  the 
other  the  upper  extremities  in  their  entirety  were  affected. 
There  were  no  sensory  symptoms  in  any  of  the  cases.  All 
signs  of  amyotrophic  lateral  sclerosis  were  absent. 


248  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

In  these  three  cases,  fourteen  years  in  the  first,  twelve 
years  in  the  second,  and  six  years  in  the  third  have  elapsed 
since  the  disease  became  quiescent.  While  in  these  cases  it  is 
by  no  means  settled  that  syphilis  was  the  cause  of  the  pro- 
gressive atrophic  paralysis,  nevertheless,  in  the  absence  of 
other  etiological  factors,  one  can  at  least  feel  that  syphilis 
furnished  the  predisposition  to  the  disease  of  the  spinal 
motor  neurons. 

Amyotrophic  Lateral  Sclerosis. — There  is  nothing  in  the 
literature  concerning  syphilis  as  a  causative  factor  of  amyo- 
trophic  lateral  sclerosis. 

Syringomyelia. — We  have  already  seen  that  isolated 
atrophic  paralyses  occur  in  syphilis,  also  that  in  spinal 
syphilis  frequently  only  individual  qualities  of  sensation, 
particularly  the  qualities  of  temperature  and  pain,  are 
affected.  It  is  not  to  be  wondered  at,  then,  if  occasionally 
the  clinical  syndrome  of  syringomyelia  is  presented.  This 
can  occur  whenever  a  syphilitic  tumor  involves  both  the 
anterior  and  the  posterior  gray  matter.  Also  it  is  known 
that  cavity  formations  occur  sometimes  both  in  cases  of 
compression  and  traumatic  myelitis. 

In  chronic  specific  myelitis  and  meningomyelitis  not  in- 
frequently one  finds  irregularly  distributed  atrophic  para- 
pareses  and  paraplegias  and  a  more  or  less  sharply  defined 
disassociation  paralysis  of  the  qualities  of  sensation.  The 
sensory  boundaries,  however,  are  of  the  same  form  as  those 
found  in  myelitis  and  not  the  characteristic  amputation  type 
occurring  in  syringomyelia.  The  development  is  also  dif- 
ferent in  that  the  disease  does  not  begin  with  atrophic 
pareses  which  gradually  progress,  followed  by  sensory 
disturbances  after  previously  the  well-known  disturbances 
of  nutrition  on  the  distal  parts  of  the  upper  or  lower  ex- 
tremities have  appeared.  In  short,  there  is  lacking  the 
regular  sequence  of  the  classical  triad  in  syringomyelia. 

In  1897  E.  Schwarz,  of  Vienna,  demonstrated  sections 
of  a  case  of  specific  myelomeningitis  with  cavity  formation 
in  the  cord.  In  the  lower  cervical  and  in  the  middle  and 
lower  dorsal  regions  in  the  anterior  horns  on  both  sides 
cavities  were  found  whose  walls  showed  no  signs  of  gliosis, 


SYPHILIS  OF  THE  SPINAL  CORD  249 

but  were  covered  with  a  thick  homogeneous  covering.  This 
homogeneous  mass  was  regarded  by  Schwarz  and  also 
Schlesinger  as  due  to  a  beginning  degenerative  process  in 
the  glia,  analogous  to  that  occurring  in  syringomyelia  and 
probably  caused  by  the  same  arterial  changes,  which  both 
Schlesinger  and  A.  Westphal  also  make  responsible  for  the 
fusing  together  of  the  gliosis-like  formation  in  syringo- 
myelia. 

In  this  case,  which  pathologically  can  be  considered  as  a 
combination  of  meningomyelitis  and  syringomyelia,  the 
clinical  symptoms  of  the  latter  were  absent. 

Central  Gliosis  with  Disease  of  the  Posterior  Columns. — 
Numerous  observers,  as  Jegorow,  Eisenlohr,  Nonne,  and 


Fig.  67.  Fig.  68. 

FIGS.  67-68. — Chronic  syphilitic  meningomyelitis.  The  myelitis  has  extended  from  the 
pia  mater  to  the  substance  of  the  cord.  (Personal  observations.)  b,  chronic  inflammatory 
proliferated  pia  mater;  a,  myelitic  changed  lateral  columns. 

Oppenheim,  have  reported  cases  with  a  combination  of 
tabetic  posterior  column  sclerosis  and  central  gliosis  with 
and  without  cavity  formation.  These  authorities  unite  in 
assuming  a  relationship  between  the  proliferation  of  the 
glia,  the  tabetic  disease  of  the  posterior  columns,  and  the 
central  gliosis,  in  that  the  gliosis  possesses  either  the  tend- 
ency to  unite  with  the  processes  of  degeneration  in  the 
posterior  columns  or  it  develops  from  the  glia-prolifera- 
tion,  appearing  secondarily  as  the  result  of  the  tabes. 

The  Brown-Sequard  Symptom-complex. — Syphilis  of  the 
spinal  cord  manifests  itself  very  often  under  the  form  of 
a  unilateral  lesion.  Petren,  in  1902,  was  able  to  collect  34 
cases  of  spinal  syphilis  which  presented  the  Brown-Sequard 
syndrome. 


250  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

When  one  stops  to  consider  the  diversity  of  specific 
lesions  which  may  affect  the  spinal  cord,  it  is  easy  to  under- 
stand the  frequency  of  occurrence  of  the  unilateral  lesion. 
It  will  also  be  easy  to  comprehend  that  the  unilateral  lesion 
will  not  be  presented  in  the  purity  in  which  we  see  it  after 
injuries,  hemorrhages,  and  non- syphilitic  tumors,  for  in 
such  conditions  the  capriciousness  of  the  specific  growth 
and  the  metamorphosis  is  absent. 

The  following  case  is  an  unusually  pure  form  of  the 
Brown-iSequard  type  of  paralysis: 

The  case  is  one  of  a  servant  girl,  twenty  years  old.  Four 
weeks  before  her  admittance  into  the  hospital  she  was 
seized  with  severe  pains  in  the  left  lower  part  of  her  body, 
which  were  girdle-like  in  character  but  radiated  chiefly  to 
the  left  side.  At  the  same  time  she  noticed  a  weakness  in 
her  right  leg.  At  the  examination  a  weakness  of  the  entire 
right  lower  extremity  and  a  diminution  in  the  pain  and 
temperature  sense  in  the  left  leg  were  found.  The  disturb- 
ance of  sensation  extended  on  the  left  side  to  the  lower  part 
of  the  abdomen  in  front  and  the  nates  behind.  Above  the 
upper  border  of  this  sensory  disturbance  there  was  a  nar- 
row zone  of  hypersesthesia.  There  was  also  a  slight  dis- 
turbance of  the  bladder  and  the  absence  of  the  patellar 
reflexes  on  both  sides. 

The  pupils  were  unequal,  did  not  react  to  light,  and  were 
slow  in  their  convergence  reaction.  The  ophthalmoscopic 
findings  were  normal.  Further  objective  symptoms  either 
in  the  internal  organs  or  nervous  system  were  not  observed. 
History  of  syphilis  was  indefinite. 

Symptomatic  therapy  failing  to  accomplish  anything, 
mercurial  inunctions  were  begun  and  from  the  fifth  day  on 
both  the  motor  and  sensory  paralysis  began  to  improve. 

The  diagrams  show  the  rate  at  which  the  sensory  dis- 
turbance receded. 

The  only  objective  symptoms  remaining  after  twenty- 
one  days  were  the  absent  patellar  reflexes. 

Eegarding  the  unilateral  lesion  Mitchell  Clark  goes  so 
far  as  to  say — in  my  opinion,  too  far — that  its  origin  is 
either  traumatic  or  syphilitic.  A  review  of  statistics  shows 


SYPHILIS  OF  THE  SPINAL  CORD 


251 


that  this  lesion  is  usually  a  chronic  one,  although  it  may 
have  an  acute  beginning.  In  the  majority  of  cases  all  the 
qualities  of  sensation  on  the  contralateral  side  are  not 


Fig.  69. 


Fig,  70. 


Fig.  71. 


Fig.  72. 


FIGS.  69-72. — 1,  left,  motor  paralysis;  right,  skin  anesthesia  for  temperature  and  pain; 
2,  seven  days  after  the  administration  of  the  inunctions;  3,  nineteen  days  afterward;  4,  twen- 
ty-one days  afterward.  Motion  and  sensation  on  both  sides  normal.  Patellar  reflexes  on  both 
sides  absent. 

affected,  and  those  involved  are  more  frequently  diminished 
than  entirely  abrogated.  The  disturbance  of  motion  is 
more  often  a  paresis  than  a  paralysis.  Corresponding  to 


252  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

the  more  frequent  localization  of  syphilis  in  the  dorsal 
cord  the  unilateral  lesion  is  confined  in  most  instances  to 
the  involvement  of  the  lower  extremities.  The  atypical 
form  of  the  syndrome  often  is  manifested  by  an  involve- 
ment also  of  the  motion  on  the  opposite  side  and  vice  versa 
disturbance  of  the  sensation  on  the  paralyzed  side. 

The  following-  sections  (Figs.  73-78)  are  taken  from  the 
cord  of  a  man,  forty  years  old,  who,  ten  years  before  the 
beginning  of  his  spinal  affliction,  contracted  lues. 

This  patient  had  a  disturbance  of  the  bladder  and  a 
slight  paresis  of  the  limbs,  with  a  suggestion  of  the  Brown- 
Sequard  type,  inasmuch  as  the  spastic  paresis  on  the  right 
side  was  more  marked  than  on  the  left  side,  and  on  the  left 
side  a  slight  hypo-SBsthesia  for  touch  and  pain  existed.  The 
symptoms  did  not  react  to  antispecific  therapy.  Death  in 
this  case  was  the  result  of  a  pneumonia. 

Experience  has  shown  us  that  cases  of  syphilis  present- 
ing this  clinical  syndrome  are  usually  benefited  and  often 
cured  by  antispecific  treatment. 

There  are  exceptions  to  this  rule,  however.  It  depends 
largely  upon  the  nature  of  the  changes  as  to  whether  they 
are  syphilitic  or  postsyphilitic. 

In  the  cases  which  recover,  or  partially  recover,  in  later 
years  a  luetic  or  metaluetic  disease  may  develop  elsewhere 
in  the  cord,  as  in  the  following  case : 

A  man  who,  two  years  before,  had  contracted  syphilis, 
was  admitted  to  the  hospital  with  a  motor  paresis  of  his 
right  limb  and  increased  patellar  reflexes.  He  complained 
of  some  pain  and  aggravating  paraesthesias  in  the  other 
limb.  Sensation  was  not  affected  in  either  leg.  These 
symptoms,  under  antispecific  therapy,  disappeared.  Two 
years  later  the  patient  again  presented  himself,  complain- 
ing of  an  insecurity  in  his  limbs.  Examination  demon- 
strated a  typical  tabes,  which  was  uninfluenced  by  treatment 
and  rapidly  progressed,  terminating  in  a  taboparesis. 

Symptoms  of  Multiple  Sclerosis. — The  picture  of  multiple 
sclerosis,  as  it  has  been  described  by  Charcot  and  as  it  is 
presented  in  typical  form  in  the  clinics,  will  scarcely  be  pro- 


SYPHILIS  OF  THE  SPINAL  CORD 


253 


Figs.  73-78. 


•*»« 


Figs.  79-83. 


FIGS.  73-78. — (Personal  observation.)  Spastic  paraplegia  inferior,  chronic  dorsal  myelitis, 
with  incomplete  Brown-Sequard. 

FIGS.  79-83. — Combination  of  incipient  tabes  with  chronic  dorsal  myelitis.  (Brown- 
Sequard.)  (Personal  observation.) 


254 


SYPHILIS  AND  THE  NERVOUS  SYSTEM 


duced  by  syphilis,  although  in  the  multiple  lesions  which  it 
causes  in  the  central  nervous  system  it  resembles  syphilis. 
The  course,  however,  is  a  different  one  and  the  symptoms, 
which  still  are  regarded  as  pathognomonic,  such  as  the 


Fios.  84-90. — Two  cases  of  combined  system  disease  in  luetics.     (Personal  ooservation.) 

scanning  speech,  oscillatory  nystagmus,  and  intentional 
tremor,  are  not  observed  in  nervous  syphilis.  Syphilis  also 
does  not  appear  to  be  a  factor  in  the  etiology  of  multiple 
sclerosis. 


SYPHILIS  OF  THE  SPINAL  CORD 


255 


The  spinal  type  of  multiple  sclerosis  (formes  frustres) 
is  often  difficult  of  differentiation  from  specific  myelitis. 
This  subject  will  be  taken  up  later  in  the  general  considera- 
tion of  the  differential  diagnosis  in  syphilis  of  the  spinal 
cord. 

Symptoms  of  Tumor. — One  can  consider  the  possibility 
of  gummatous  tumor  of  the  spinal  cord  in  those  cases  in 
which  the  Brown-Sequard  syndrome  is  well  developed  or 
where  an  increasing  compression  of  the  cord  exists  along 
with  severe  symptoms  of  sensory  irritation,  or  where  a 
circumscribed  subacute  anterior  poliomyelitis  develops. 
Such  cases  have  been  described  by  Osier,  Orlowsky,  Gowers, 
and  Williamson.  The  tumor  symptoms  in  these  cases  are 


Fia.  91. 

naturally  the  same  as  are  caused  by  other  tumors,  as,  for 
example,  an  intramedullary  tubercle  reported  by  Sachs.  It 
must  be  remembered,  however,  that  these  tumor  symptoms 
can  be  produced  by  the  cone-shaped  gummatous  prolifera- 
tions which  penetrate  into  the  cord,  also  that  chronic  myelo- 
meningitis  cannot  be  differentiated  from  circumscribed 
gummatous  tumors,  because  with  the  tumors  either  local- 
ized or  diffuse  meningitis  is  always  an  accompaniment. 
In  general,  one  can  think  of  the  possibility  of  a  gumma 
whenever  the  symptoms  of  an  extra-  or  intramedullary 
tumor  are  presented. 

Symptoms  of  Combined  Tract  Disease. — One  appreciates 
a  priori  that  disease  of  the  vessels  in  spinal  lues  through 
their  different  localizations  can  cause  the  clinical  syndrome 


256  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

of  combined  tract  disease,  and  also  that  meningitis  involv- 
ing the  cord  secondarily  may  attack  the  posterior  and  lat- 
eral columns  in  different  combinations. 

Our  experience  in  " syphilitic  spinal  paralysis"  has 
taught  us  that  the  primary  combined  system  disease  in  the 
form  of  degeneration  of  the  cross-pyramidal  tract  and  other 
tract  systems  in  the  lateral  columns,  together  with  disease 
of  the  columns  of  (roll,  occurs. 

The  cross-pyramidal  tracts  may  also  degenerate  pri- 
marily alone. 

The  formerly  generally  accepted  view  that  syphilis  does 
not  cause  system  disease,  in  the  light  of  our  present  knowl- 
edge, does  not  hold  good. 

What  Pierre  Marie  calls  the  vascular  form  of  system 
disease  is  by  far  the  most  frequent,  a  pseudosystem  affec- 
tion developing  from  an  existing  localizing  meningo- 
myelitis. 

The  following  case  is  such  a  one  reported  by  Dreschfeld 
in  1888: 

A  patient,  sixteen  and  a  half  months  after  a  specific 
infection,  while  he  still  presented  secondary  symptoms,  de- 
veloped a  spastic  paraparesis  of  the  lower  extremities, 
which  were  ataxic  and  also  showed  slight  sensory  disturb- 
ances. The  bladder  was  also  paralyzed.  The  upper  ex- 
tremities were  not  involved.  Some  time  after  antispecific 
therapy  had  caused  a  marked  improvement,  the  patient  died 
from  a  cystopyelitis. 

The  pathological  examination  demonstrated  a  diffuse 
sclerosis  of  the  posterior  lateral  and  anterior  pyramidal 
tracts,  as  well  as  of  the  gray  matter  in  the  lower  portion 
of  the  dorsal  cord.  At  the  same  time  there  was  an  ascend- 
ing degeneration  of  the  column  of  Goll  and  a  partial  one 
of  Burdach's  column  and  the  lateral  cerebellar  tracts.  In 
the  sclerotic  changes  there  was  a  well-developed  peri-  and 
endoarteritis  and  perivascular  infiltration.  The  pia  was 
not  affected. 

Recently  Renner  published  a  case  with  the  pathological 
examination.  There  existed,  after  prodromal  pains  in  the 
limbs,  ataxia  in  the  arms,  spastic  paresis  in  the  lower  ex- 


SYPHILIS  OF  THE  SPINAL  CORD  257 

tremities,  weakness  of  the  bladder  with  cystitis,  loss  of  the 
pupil  reaction  to  light,  optic  atrophy,  and  slight  girdle-like 
sensory  anomalies  on  the  body.  Clinically  there  appeared 
to  be  a  beginning  cervical  tabes  with  a  spastic  spinal 
paralysis.  There  was  found  pathologically  a  combined 
tract  affection,  which  in  the  cervical  cord  involved  a  part  of 
the  posterior  columns  and  the  cross-pyramidal  tracts,  in 
the  rest  of  the  cord  only  the  cross-pyramidal  tracts. 

Pupil  anomalies  also  sometimes  occur  along  with  symp- 
toms of  posterior  and  lateral  column  disease. 

In  such  cases,  from  our  knowledge  gained  in  autopsy 
findings,  we  may  assume  a  primary  nuclear  degeneration 
in  the  oculomotor  nucleus,  in  addition  to  the  specific 
meningomyelitis  causing  posterior  column  symptoms. 

Another  combination  is  that  of  cross-pyramidal  tract 
symptoms  and  rudimentary  forms  of  tabes,  which  present 
the  picture  of  primary  optic  atrophy,  loss  of  the  pupil  reac- 
tion to  light,  lancinating  pains,  and  slight  bladder  disturb- 
ances, also  occasionally  disturbances  of  sensation. 

A  man  who,  ten  years  before,  had  been  treated  for  pri- 
mary and  secondary  syphilis,  came  under  my  observation. 
For  two  years  he  has  had  a  double  primary  optic  atrophy, 
at  the  same  time  lancinating  pains  in  the  feet,  and  a  slight 
disturbance  of  the  bladder.  The  examination  showed,  in 
addition  to  the  optic  atrophy,  which  was  tabetic  in  form, 
increase  in  the  tendon-reflexes,  and  slight  spastic  paresis  of 
the  lower  extremities.  There  was  no  ataxia,  no  sensory 
disturbances  and  no  Romberg.  The  pupils  were  unequal  in 
size  and  reacted  slowly  to  light. 

After  five  years  an  examination  of  this  patient  revealed 
that  the  optic  atrophy  had  become  complete,  the  spastic 
condition  of  the  lower  extremities  remained  unchanged,  and 
no  new  symptoms  had  appeared.  Cases  of  the  above  type 
are  not  infrequent. 

Spinal  Roots. — There  still  remain  a  few  words  to  be  said 
concerning  the  symptoms  caused  by  the  spinal  roots. 

The  meningeal  symptoms  are  essentially  root  symptoms, 
the  hyperaesthesia  of  the  back,  the  girdle-like  pains,  the 
neuralgic  sensations  radiating  into  the  extremities,  are 

17 


258  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

caused  by  irritation  of  the  posterior  sensory  roots,  and 
these  are  irritated  in  turn  by  the  thickened  and  inflamed 
cord-membranes  through  which  they  must  pass.  The  symp- 
toms of  irritation  on  the  part  of  the  anterior  motor  roots 
consist  in  twitching,  fine  tremor,  and  in  atrophic  paresis 
and  paralysis. 

Clinically  it  is  impossible  to  differentiate  whether  these 
root  symptoms  are  caused  by  a  root  neuritis  which  is  inde- 
pendent of  the  meningitis,  or  by  the  meningitis  and  its  con- 
sequent effect  upon  the  roots. 


XIII 

TABES  AND  SYPHILIS 

The  Relationship  of  Tabes  and  Syphilis. — The  relationship 
between  syphilis  and  tabes  is  such  an  intimate  one  that, 
although  a  description  of  the  clinical  symptoms  of  the 
former  does  not  enter  into  our  theme,  it  is  important  that 
this  relationship  receives  thorough  discussion. 

Historical. — Since  Fournier,  in  1875,  asserted  that  tabes 
was  the  result  of  syphilis,  two  opinions,  one  which  affirms, 
and  the  other  which  denies,  have  been  sharply  contrasted. 

In  France  Vulpian  and  Grasset  soon  accepted  Four- 
nier's  view,  while  Charcot,  on  the  other  hand,  combated  it 
to  the  last.  In  England  Gowers  was  one  of  the  first  to 
become  a  supporter  of  the  Fournier  teaching.  In  Germany 
Erb  early  became  the  most  ardent  advocate  of  this  doc- 
trine. At  the  head  of  the  opponents  in  Germany  stand 
C.  Westphal  and  Leyden. 

Arguments  for  the  Relationship. — Those  who  assume  an 
etiological  relationship  between  the  two  diseases  give  the 
following  reasons  for  their  position : 

First.  In  the  clinical  course  of  tabes  and  the  syphilitic 
diseases  of  the  central  nervous  system  numerous  analogies 
are  to  be  found.  One  often  finds  in  both  affections  paralyses 
of  the  eye  muscles,  which  in  both  show  a  fleeting  character. 
In  both  the  optic  nerve  is  often  involved,  and  pupil  anoma- 
lies and  absence  of  the  patellar  reflexes  frequently  occur. 

Second.  Statistics  show  that  the  majority  of  tabetics 
have  had  syphilis.  The  height  of  the  percentage  varies 
with  the  different  authorities.  Erb  in  900  cases  of  tabes 
was  able  to  determine  a  past  syphilis  in  90  per  cent,  of  the 
cases,  and  in  a  later-collected  series  of  1100  cases  taken 
from  the  higher  classes  89.45  per  cent,  were  found  to  have 
had  a  syphilitic  infection.  In  this  series  62.9  per  cent,  had 
a  history  of  secondary  symptoms,  and  26.54  per  cent,  gave 
a  history  of  a  soft  chancre.  This  leaves  only  10.54  per  cent, 
in  which  no  infection  could  be  demonstrated. 

259 


260  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

Founder's  view  which  gave  the  impetus  to  the  syphilis- 
tabes  conception  was  based  originally  on  30  cases  in  which 
a  history  of  syphilis  was  obtainable  in  24. 

The  wide  variation  in  the  percentage  may  be  seen  in 
the  statistics  from  different  observers.  Berger  found  a 
history  of  a  previous  syphilis  in  20  per  cent,  of  his  cases, 
Growers  in  53  per  cent.,  Voight  in  67  per  cent.,  Seelig- 
muller  and  Genesius  in  28  per  cent.,  Eehlen  in  23  per  cent., 
and  Westphal  in  only  14  per  cent. 

With  the  increase  of  interest  and  discussion  of  this 
question  it  is  surprising  to  observe  how  the  percentage  in- 
creased. For  instance,  Eulenburg  in  1878  reported  149 
cases  of  tabes  in  which  there  was  only  one  that  he  regarded 
constitutional  syphilis  as  the  probable  cause,  and  again  in 
1885  discovered  a  venereal  infection  in  36.8  per  cent.,  of 
which  percentage  66  per  cent,  were  undoubtedly  syphilitic. 
The  expression  of  other  observers  was  the  same.  Berger 's 
statistics  went  from  20  to  43  per  cent.,  Bernhardt  's  from  21 
to  83  per  cent.,  Voight 's  from  67  to  82  per  cent.,  and  Oppen- 
heim's  from  17  to  80  per  cent. 

The  following  statistics  are  taken  from  Erb 's  review : 


Previous  Syphilis  in  Tabes. 

France. 


Per  Cent. 

Fournier 93 

Labbe 93 

Martineau . .         95 


Per  Cent. 


Dejerine 97 

Ferras 91 

Belugou  and  Faure 77 


Germany  and  Austria. 


Voight 82 

Rumpf 85 

G.  Fisher,  certain  72  per  cent.,  prob- 
able...   90 

Seeligmann 86 

Eisenlohr 60 


Bernhardt 83 

Remak 63.5 

Strumpell 90 

Hirt 92 

Kuhn  (Jolly's  clinic) 81.6 


England. 


Gowers 75  to  80 

Althaus..  86.5 


Bramwell 76 

Mott . .  .70 


America. 


Seguin 72 

Spitzka 80 


Dana 68 

Collins  . .  .80 


Bonar 69 

Variation  in  Statistics. — How  at  variance  the  statistics 
on  this  question  may  be  depends  very  much  upon  which  side 
of  the  argument  one  is,  even  when  those  statistics  are  from 
the  same  city  and  hospital,  and  published  at  the  same  time 


TABES  AND  SYPHILIS  261 

by  conscientious  and  capable  observers,  as  was  the  case 
in  the  statistics  of  Kuhn  and  Guttmann.  Kuhn's  material 
was  taken  from  Jolly's  clinic  in  the  Charite,  Guttmann 's 
from  Leyden's  clinic  in  the  same  hospital,  the  former  is  a 
supporter  of  the  syphilis-tabes  teaching,  and  the  latter  an 
opponent.  Guttmann,  in  25  cases  of  tabes  which  he  collected 
from  the  files  of  a  life  insurance  company,  was  unable  to 
find  in  the  history  any  evidence  of  a  past  syphilis  in  a  single 
case,  while  a  glance  at  Kuhn's  statistics  in  the  table  above 
shows  a  history  of  syphilis  in  81.6  per  cent,  of  his  cases. 

The  opponents  of  this  doctrine  have  recently  proposed 
the  query  as  to  what  proportion  of  syphilitics  develop 
tabes.  The  question  is  a  difficult  one  to  answer,  because 
there  is  no  official  requirement  for  reporting  luetic  cases, 
and  no  one  knows  how  many  persons  there  are  who  have 
been  infected  with  syphilis.  Eeumont  has  found  1.6  per 
cent,  of  tabetics  in  his  experience,  Matthes  1  per  cent.,  and 
Erb  estimates  from  2  to  5  cases  of  tabes  in  every  100  cases 
of  syphilis. 

Sarbo  in  27,813  patients  belonging  to  a  sick-benefit 
society  in  Budapest,  among  whom  syphilis  was  very  com- 
mon, found  tertiary  lues  in  31  patients,  cerebrospinal  lues 
in  17,  and  tabes  in  34. 

Hudovernig  and  Guszmann  from  statistics  in  50  cases 
of  syphilis,  in  which  the  infection  dated  back  more  than 
3  years,  came  to  a  surprising  result  for  the  opponents  in 
this  controversy.  In  their  cases  where  tertiary  syphilis 
was  present  a  normal  nervous  system  was  found  in  only 
44  per  cent.,  while  on  the  other  hand  tabes,  taboparesis,  and 
general  paresis  was  found  in  46  per  cent. 

Conjugal  Tabes. — Not  infrequently  tabes  has  been  ob- 
served in  both  husband  and  wife,  and  in  all  these  cases  the 
history  of  syphilis  was  positive.  I  have  had  the  oppor- 
tunity of  observing  many  cases  of  conjugal  tabes,  and 
among  these  cases  there  was  only  one  in  which  lues  could 
not  be  demonstrated  in  the  history,  and  later  at  the  autopsy. 
In  addition  to  this  experience  of  mine  I  have  been  able  to 
find  in  the  literature  only  one  case  of  conjugal  tabes  where 
syphilis  was  lacking. 


262 


SYPHILIS  AND  THE  NERVOUS  SYSTEM 


Concerning  the  frequency  of  conjugal  tabes  the  statis- 
tics of  Monkemoller  and  Heubner  may  be  consulted. 
Monkemoller  in  741  cases  of  paresis  found  18  conjugal 
cases.  Heubner  in  450  cases  of  paresis  and  tabes  in  mar- 
ried women  found  14  conjugal  cases. 

The  following  tables  are  taken  from  my  own  experience 
in  hospital  and  private  practice. 

In  the  seven  years,  from  1901  to  1907,  there  were  200 
cases  of  paresis  admitted  to  my  department  at  Eppendorf, 
of  which  number  156  were  men  and  44  women.  In  these 
200  cases  it  was  possible  in  74  to  examine  both  husband  and 
wife.  During  this  same  period  103  cases  of  tabes  were  also 
admitted,  of  which  number  66  were  men  and  37  women.  In 
these  103  cases  both  the  husband  and  wife  were  examined 
in  76. 

TABLE  I 


Paresis. 

Tabes. 

Men  

.  156 

Men  

.  66 

Women.  .  . 

.      •      44 

Women  

37 

200  (74) 


103  (76) 


303  (150) 

In  the  74  cases  of  paresis  in  which  both  husband  and 
wife  were  examined  undoubted  conjugal  paresis  was  found 
in  6  cases  and  suspicious  symptoms  in  7  cases. 

It  was  a  surprising  fact  in  the  cases  in  which  the  wife 
was  brought  in  with  paresis  and  then  the  husband  was  later 
examined  that  the  conjugal  disease  was  much  oftener  en- 
countered than  when  the  husband  was  the  primary  patient. 
Exactly  the  same  situation  was  also  found  to  be  true  with 
reference  to  tabes. 

TABLE  II 


Primary. 

Conjugal. 

Suspicious. 

Primary. 

Conjugal. 

Suspicious. 

Husband      56 

1 

6 

Males  45 

9 

Wife  18 

5 

1 

Females.  .  .31 

12 

2 

74 

6 

7 

76 

21 

2 

Cases  of  paresis  and  tabes  with  primary  disease  of  the  husband .  101.   Conjugal  10 
Cases  of  paresis  and  tabes  with  primary  disease  of  wife 39.   Conjugal  17 

Total  number  of  cases  of  paresis  and  tabes  in  man  and  wife .  150.   Conjugal  27 


TABES  AND  SYPHILIS  263 

The  only  explanation  for  this  remarkable  relationship 
of  both  tabes  and  paresis  in  the  rare  occurrence  of  either 
tabes  or  paresis  in  married  women  whose  husbands  have 
primarily  one  or  the  other  of  these  diseases  lies  in  the  fact 
that  the  husband  who  contracted  his  infection  earlier  in 
life,  before  his  marriage,  usually  has  passed  by  the  stage 
where  he  is  able  to  transmit  the  infection. 

The  occurrence  of  tabes  and  paresis  and  other  syphilo- 
genetic  organic  nervous  diseases  in  families  has  been  dis- 
cussed in  a  former  chapter. 

The  Late  Development  of  Tabes. — In  those  cases  in  which 
tabes  appeared  unusually  late  in  life  the  syphilis  was  con- 
tracted unusually  late.  Numerous  cases  of  this  nature  have 
been  reported. 

In  a  case  which  came  under  my  observation  the  patient 
acquired  his  syphilis  in  his  fifty-sixth  year,  and  in  his  six- 
tieth year  presented  the  symptoms  of  primary  optic 
atrophy,  lightning  pains,  Argyll-Robertson  pupils,  loss  of 
the  patellar  reflexes,  and  hypalgesia — in  short,  the  picture 
of  a  classical  tabes. 

In  a  second  case  the  patient  was  sixty-six  years  old,  and 
had  been  infected  with  syphilis  when  he  was  sixty.  He  be- 
gan in  his  sixty-fourth  year  with  a  primary  optic  atrophy, 
and  at  his  examination,  two  years  later,  I  was  able  to  demon- 
strate Argyll-Robertson  pupils,  Romberg's  symptom,  loss 
of  the  patellar  reflexes,  and  characteristic  sensory  dis- 
turbances on  the  back  and  lower  extremities. 

The  Frequency  of  Sterility  in  Tabetics. — Mendel  in  252 
married  women  with  tabes  who  were  examined  by  him  found 
that  32.9  per  cent,  were  childless. 

The  absence  of  offspring  depends  either  upon  the  fact 
that  conception  has  not  occurred,  or  that  the  child  died  soon 
after  birth,  usually  within  the  first  month,  or  that  abortion 
occurred.  The  percentage  of  childless  marriages  in  gen- 
eral has  been  estimated  at  from  10  to  15  per  cent.,  so  that 
it  will  be  seen  that  childlessness  in  tabetic  women  is  almost 
three  times  as  frequent  as  in  non-tabetic,  living  under  the 
same  social  conditions. 

The  occurrence  of  tabes  and  arteriosclerotic  aneurism 


264  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

of  the  aorta  together  has  been  frequently  observed  at  com- 
paratively an  early  age.  I  have  observed  six  cases  of  this 
character,  and  in  all  of  them  except  one  lues  could  be 
demonstrated. 

Concerning  the  frequency  of  tabes  and  disease  of  the 
heart  and  aorta,  Striimpell  has  recently  directed  our  atten- 
tion. His  deductions  were  as  follows: 

(a)  In  patients  with  insufficiency  of  the  aortic  valves, 
sclerosis  of  the  aorta,  and  aortic  aneurism  one  finds  not 
infrequently,  when  carefully  sought  for,   signs  of  tabetic 
disease. 

(b)  Well-marked  tabes  is  often  found  in  combination 
with  aortic  insufficiency  and  aortic  sclerosis. 

(c)  This  combination  of  both  diseases  is   simply  the 
consequence  of  a  previous  syphilis. 

Tabes  in  Hereditary  Lues. — Tabes  occurs  in  children  with 
hereditary  lues  and  also  in  adults  as  a  later  expression  of 
hereditary  syphilis,  just  as  the  infantile  or  juvenile  paresis 
develops  after  hereditary  syphilis. 

Tabes  in  children  naturally  has  a  particular  value  in  the 
determining  of  the  etiology  of  the  disease,  since  the  other 
causative  factors,  as  trauma,  alcoholism,  psychic  trauma, 
overexertion,  and  sexual  excesses  may  be  eliminated. 

In  five-sixths  of  these  cases  syphilis  can  be  definitely 
proven  (Marburg).  In  striking  contrast  to  tabes  in  adults 
after  acquired  lues  the  disease  is  equally  distributed  be- 
tween the  sexes,  which  may  be  explained  by  the  fact  that 
hereditary  syphilis  of  course  does  not  favor  either  sex, 
while  in  acquired  syphilis  men  are  more  often  affected 
than  women. 

The  occurrence  of  tabes  and  paresis  is  not  so  rare.  In 
my  own  experience  I  have  found  tabetic  children  in  ten 
different  families.  In  all  these  cases  there  was  a  history 
of  syphilis  in  the  family,  in  nine  cases  either  the  father  or 
mother  or  both,  in  one  case  the  child  was  infected  extra- 
genitally.  I  do  not  know  of  a  single  case  of  infantile  or 
juvenile  paresis  or  tabes  without  syphilis. 

Tabes  and  Syphilitic  Disease  in  the  Same  Family. — I  have 
observed  a  number  of  times  that  members  of  the  same 


TABES  AND  SYPHILIS  265 

family  (brother  and  sister)  who  had  become  infected  with 
syphilis  developed  disease  of  the  central  nervous  system. 
In  one  instance  two  brothers,  the  one  several  years  after 
his  infection  developed  paresis,  the  other  tabes.  In  two 
other  families  both  brothers  in  each  family  became  tabetic. 
In  another  family  one  brother  was  affected  with  syphilitic 
meningomyelitis,  as  the  autopsy  proved,  and  the  other  with 
a  typical  tabes.  In  still  another  family  one  brother  was 
tabetic  and  the  other  was  affected  with  Erb's  syphilitic 
spinal  paralysis. 

The  cases  which  Erb  presented  to  the  International 
Congress  at  Moscow  are  very  pertinent  here.  One  case 
concerned  three  brothers  who  had  suffered  a  psychic 
trauma,  two  who  had  never  had  syphilis  developed  neuras- 
thenia, while  the  third  brother,  who  was  a  syphilitic,  became 
affected  with  tabes.  In  another  case  a  man  who  had  been 
a  syphilitic  instead  of  having  regular  sexual  intercourse 
with  his  wife  practised  onanistic  manipulations;  the  wife 
developed  hysteria,  the  man  tabes. 

Combination  of  Tabes  with  Syphilitic  Disease  of  the  Ner- 
vous System  and  Internal  Organs. — The  supporters  of  the 
Fournier-Erb  doctrine  admit  that  the  pathology  of  tabes  is 
different  from  that  of  syphilis,  and  that  also  the  disease  of 
the  vessels  in  tabes  is  not  a  luetic  one.  In  short,  the  pa- 
thology in  cases  of  tabes  with  a  history  or  other  evidence  of 
syphilis  does  not  differ  in  any  way  from  the  cases  in  which 
the  history  or  other  evidence  of  lues  is  entirely  lacking. 

Adrian  has  made  a  comprehensive  and  thorough  study 
of  the  occurrence  of  visible  syphilis  and  tabes  at  the  same 
time.  He  has  divided  his  cases  into:  three  groups : 

1.  Cases  with  pathological  findings  of  tabes  with  also 
active  evidences  of  syphilis  in  the  central  nervous  system. 
He  reports  16  cases  in  this  group,  to  which  I  am  able  to 
add  4  more  (3  cases  of  brain  gummata  and  1  case  of  specific 
meningitis  along  with  beginning  tabes). 

2.  Pathological    findings    of    active    syphilis    in    other 
organs  outside  of  the  central  nervous  system,  and  evidences 
of  tabes  in  the  nervous  system.    Adrian  reports  15  cases 
here,  to  which  I  can  add  7. 


266  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

3.  Cases  of  tabes  in  which  active  manifestations  of 
syphilis  have  been  observed  during  life  in  conjunction  with 
tabes  are  by  far  the  largest  group.  Adrian  reports  in  this 
group  65  cases. 

As  a  result  of  his  study  of  these  groups  Adrian  comes 
to  the  following  conclusions,  which  I  believe  to  be  correct: 

1.  That  the  syphilitic  virus,  although  latent,  in  combina- 
tion with  well-marked  tabes  may  exist  for  years  in  the 
system.    • 

2.  A  combination  of  the  visible  manifestations  of  syph- 
ilis on  the  skin  or  in  the  internal  organs  and  a  typical  tabes 
is  not  so  rare  as  it  was  formerly  supposed  to  be. 

3.  Concerning  the  frequency  of  the  coexistence  of  these 
two  conditions  nothing  definite  at  the  present  time  can 
be  said. 

A  careful  examination  of  patients  with  tertiary  syphilis 
for  evidence  of  tabes  should  always  be  made. 

4.  The  coexistence  of  active  syphilitic  changes  in  the 
various  internal  organs  and  tabes  compels  us  to  the  assump- 
tion of  a  close  relationship  between  the  two  diseases. 

A  Combination  of  Specific  Spinal  Meningitis  and  Tabes. — 

The  question  as  to  whether  the  tabetic  changes  in  the  pos- 
terior columns  of  the  cord  are  the  result  of  the  spinal  menin- 
gitis in  the  cases  reported  has  been  carefully  considered, 
and  the  conclusion  arrived  at  that  the  tabes  was  entirely 
independent  of  the  meningitis.  The  observers  came  to  this 
conclusion  because  the  meningeal  changes  at  the  different 
cord  levels  did  not  correspond  to  the  degenerations  of  the 
posterior  columns,  and  because  also,  while  the  anterior 
roots  were  surrounded  equally  with  the  posterior  roots 
with  meningeal  proliferations,  the  anterior  roots  were  not 
degenerated. 

Striimpell's  Toxin  Theory. — The  present  standpoint  of  the 
majority  of  the  supporters  of  the  tabes-syphilis  doctrine 
is  the  one  which  Striimpell  first  proposed.  Striimpell  pro- 
pounded the  theory  that  syphilis  produces  a  toxin  which 
has  an  injurious  effect  upon  the  ganglion-cells  and  nerve- 
fibres  of  the  nervous  system.  The  action  of  this  toxin  does 


TABES  AND  SYPHILIS  267 

not  refer  to  the  direct  consequences  of  syphilis  which  pro- 
duces characteristic  pathological  changes,  but  only  to  an 
indirect  or  secondary  action  which  one  can  imagine  as  due 
to  the  discharge  of  a  chemical  poison.  Strumpell  calls  atten- 
tion to  the  severe  clinical  nervous  disturbances  following 
diphtheria  in  exemplifying  his  theory,  the  relation  of  which 
to  the  toxin  of  diphtheria  no  one  doubts.  The  opponents  of 
the  Fournier-Erb  teaching  on  the  other  hand  say  that  this 
theory  is  purely  an  arbitrary  one,  and  that  nothing  is  known 
of  a  toxin  of  syphilis. 

Without  the  assumption  of  a  toxin  effect,  however, 
some  cases  would  be  difficult  to  explain,  as  for  instance 
the  disease  of  the  spinal  cord  in  severe  anaemias  and  in 
carcinoma.  In  accepting  Strumpell 's  theory  one  must  of 
course  admit  a  selective  action  on  the  sensory  portion  of 
the  cord  by  the  syphilitic  toxin.  This  ought  not  to  be  diffi- 
cult because  we  have  been  aware  for  many  years  of  the 
selective  action  of  poisons  on  the  nervous  system.  We 
know  for  example  that  lead  has  a  predilection  for  the  motor 
and  ergot  for  the  sensory  tracts,  and  that  the  toxin  which 
is  produced  in  pernicious  anaemia  injures  chiefly  the  pos- 
terior columns. 

The  Position  of  the  Opponents  in  the  Tabes-syphilis  Dis- 
cussion.— The  opponents  of  the  doctrine  that  tabes  and 
syphilis  are  etiologically  related,  among  whom  are  Leyden 
and  Goldschneider,  deny  the  affirmative  value  of  statistics 
and  bring  statistics  to  prove  the  opposite  opinion. 

It  has  been  admitted  by  Erb  himself  and  also  by  Hitzig, 
Mendel,  Kron,  and  others  that  in  every  series  there  is 
always  a  number  of  cases  remaining  in  which  either  syph- 
ilis cannot  be  demonstrated  or  at  least  is  extremely  improb- 
able. It  must  be  confessed  that  it  is  somewhat  questionable 
to  assume  in  these  cases,  as  Mobius  does,  that  also  here  a 
specific  infection  has  existed  but  that  its  proof  has  escaped 
our  knowledge.  Erb  says  in  his  last  monograph  concern- 
ing tabes,  published  in  1905,  that  at  the  present  time  it  can- 
not be  absolutely  proven  that  tabes  without  exception  in 
all  cases  is  a  syphilogenetic  disease.  Personally  I  can  recall 


268  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

many  cases  in  which  syphilis,  with  the  certainty  which  is 
permitted  in  our  assumptions  and  conclusions,  could  be 
excluded. 

The  chief  arguments  of  the  opponents  along  with  the 
garbling  of  the  statistics  are,  first,  that  the  pathology  of 
true  tabes  shows  nothing  of  syphilis,  and,  second,  that  anti- 
specific  treatment  is  useless. 

The  first  argument  falls  to  the  ground  when  one  con- 
siders it  from  Striimpell's  view-point  and  regards  tabes 
simply  as  the  result  of  the  indirect  action  of  lues.  Since 
tabes  cannot  be  regarded  as  an  expression  of  syphilitic 
cachexia,  but  on  the  contrary  usually  begins  in  the  prime 
of  life  in  robust  individuals,  one  cannot  compare  it  with 
amyloid  degeneration  as  Virchow  does  and  demand  in  old 
luetic  cases  where  this  type  of  degeneration  occurs  so  often 
that  tabes  should  be  found  more  frequently. 

Marie's  Theory. — In  1903  Pierre  Marie  together  with  Guil- 
lain  presented  the  view  pathologically  of  tabes  that  it  was  a 
syphilis  of  the  meninges.  According  to  their  conception 
it  was  the  posterior  part  of  the  pia  alone  which  underwent 
inflammatory  changes.  The  pathological  process  of  tabes 
consists  in  a  lesion  of  the  posterior  roots,  and  a  posterior 
meningitis  which  pathologically  possesses  all  the  ear-marks 
of  a  specific  meningitis.  According  to  Marie  there  exists 
in  the  pia  mater  a  lymph  system.  The  cell  elements  which 
are  found  in  the  spinal  fluid  of  tabetics  are  lymph-cells. 
Histology  teaches  us  that  the  lymph-system  of  the  posterior 
part  of  the  pia  mater  scarcely  communicates,  or  communi- 
cates not  at  all,  with  the  lymph  system  of  the  anterolateral 
portion  of  the  pia. 

The  pathology  of  the  posterior  part  of  the  pia  is  a 
separate  pathology  in  itself.  The  tabetic  process  consists 
in  a  lesion  of  the  entire  posterior  lymph  system  of  the  cord. 
According  to  Marie's  theory  then  tabes  is  a  syphilitic  dis- 
ease of  this  lymph  system.  According  to  the  consensus  of 
opinion  of  the  majority  of  the  authorities  on  this  subject 
simplicity  is  about  the  only  recommendation  that  can  be 
made  for  Marie's  theory. 


TABES  AND  SYPHILIS  269 

The  value  of  the  almost  constant  appearance  of  excess 
globulin  and  lymphocytosis,  as  well  as  the  Wassermann 
reaction  in  the  spinal  fluid  in  both  tabes  and  paresis,  as  an 
argument  in  favor  of  their  syphilogenetic  origin,  will  be 
discussed  in  a  special  chapter. 

The  Antispecific  Therapy  as  an  Argument  is  Useless. — 
The  argument  against  the  specific  origin  of  tabes  and 
paresis  because  antispecific  treatment  has  no  influence  on 
these  diseases  will  not  bear  the  light  of  reason.  Diphthe- 
ritic paralysis  is  also  uninfluenced  by  the  administration  of 
antidiphtheritic  serum.  The  cirrhosis  of  the  liver  produced 
by  the  chronic  use  of  alcohol  remains  incurable  even  when 
the  use  of  alcohol  has  been  stopped.  The  neuritis  pro- 
duced by  malaria  is  in  no  way  influenced  by  quinine.  My 
position  in  the  treatment  of  tabes  with  mercury  will  be 
made  clear  in  the  chapter  on  therapy.  It  is  sufficient  to  say 
here  that  I  have  never  seen  a  case  of  tabes  cured  or  a  fully 
developed  case  even  materially  improved  by  mercurial 
treatment.  It  is  another  question,  however,  to  say  whether 
in  the  beginning  of  the  disease  mercury,  if  administered  in 
an  intelligent  manner,  may  not  exert  a  restricting  and  re- 
tarding influence  on  its  course.  Dinkier 's  experience  in 
Erb's  clinic,  and  Erb's  own  statements,  cause  one  at  any 
rate  to  reflect  seriously  on  this  question.  My  own  observa- 
tions in  this  regard  have  shown  a  long  series  of  cases  of 
tabes  incompleta,  or  tabes  imperfecta,  abortiva,  rudimen- 
tary, or  whatever  nomenclature  one  chooses  to  adopt  for 
such  cases,  all  with  a  history  of  syphilis  in  the  anamnesis, 
which  were  treated  for  a  number  of  years  in  succession 
with  mercury  and  tonic  measures  and  remained  in  their 
rudimentary  and  imperfectly  developed  condition. 

The  Infrequency  of  Tabes  in  Prostitutes. — The  inf requency 
of  tabes  in  prostitutes  as  another  argument  for  the  oppo- 
nents of  the  syphilis-tabes  theory  is  that  prostitutes  very 
often  contract  syphilis  but  seldom  develop  tabes.  The 
argument  appears  less  convincing  when  one  considers  that 
many  prostitutes  die  young,  of  either  pulmonary  tubercu- 
losis or  alcoholism,  and  that  many  more  give  up  their 
occupation  at  an  age  when  tabes  is  most  likely  to  appear, 


270  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

and  if  later  they  become  tabetic  are  apt  to  remain  silent 
regarding  their  former  life.  The  statistics  of  Kron,  who 
found  14  per  cent,  of  prostitutes  who  were  in  the  tabes- 
developing  age  to  be  tabetic,  are  a  large  percentage.  Heub- 
ner  found  in  179  prostitutes  9.9  per  cent,  tabetic.  The  same 
observer  also  found  among  prostitutes  dying  in  institutions 
that  58.5  per  cent,  died  of  paresis,  5  per  cent,  of  tabes,  and 
24  per  cent,  of  cerebrospinal  lues.  Jadassohn  has  stated 
that  according  to  his  experience  tabes  among  prostitutes  is 
of  frequent  occurrence,  and  Kraepelin  says  that  paresis  is 
remarkably  common  in  young  women  who  are  prostitutes. 

The  Misproportion  Between  the  Frequency  of  Syphilis  and 
the  Infrequency  of  Tabes  in  Different  Countries. — The  statis- 
tics on  this  subject  have  again  proven  themselves  to  be 
unreliable.  One  set  of  observers  produce  statistics  showing 
that  syphilis  is  wide-spread  in  such  countries  as  central 
Asia,  India,  Japan,  Abyssinia,  Turkey,  and  among  the  col- 
ored population  in  the  various  countries  where  they  are 
found,  while  tabes  is  exceedingly  rare.  Another  set  of 
observers  on  the  other  hand  prove  by  statistics  exactly  the 
reverse  to  be  true.  For  instance,  Jeanselme  has  stated  that 
in  Indo-China  syphilis  is  exceedingly  wide-spread  and  also 
that  gummatous  diseases  of  the  brain  and  spinal  cord  occur 
not  infrequently,  while  on  the  other  hand  parasyphilitic 
affections  are  entirely  unknown. 

Halban  on  the  contrary  finds  that  tabes  in  Abyssinia  is 
six  times  as  frequent  as  it  is  in  Vienna;  Collins,  of  New 
York,  finds  only  one-fourth  as  many  cases  of  tabes  there 
proportionately  as  Holzinger  finds  in  Abyssinia,  and  Hecht 
reports  tabes  as  by  no  means  uncommon  in  the  American 
negro. 

It  is  interesting  to  note  that  while  Gliick  from  his  experi- 
ences became  an  opponent  of  the  syphilis-tabes  theory, 
Hodlmoser,  who  worked  in  the  same  city  (Sarajewo, 
Bosnia)  and  indeed  in  the  same  hospital,  was  an  ardent 
supporter  of  it.  Both  Diihring  and  Gliick  have  called  atten- 
tion to  the  fact  that  in  their  material  not  only  tabes  but  also 
all  specific  nervous  diseases  were  exceedingly  rare.  Diihr- 
ing states  according  to  his  experience,  in  general,  this  is 


TABES  AND  SYPHILIS  271 

due  to  the  nature  of  the  syphilis,  and,  in  comparison  to  the 
lesions  of  the  mucous  membrane  and  skin,  the  nervous 
system  is  very  seldom  affected.  In  other  words,  the  lues 
found  in  Turkey,  Bosnia,  and  Asia  Minor  with  reference 
to  its  action  on  the  nervous  system  possesses  an  entirely 
different  influence  from  the  ordinary  syphilis  occurring  in 
Europe. 

In  this  connection  the  well-known  fact  will  become  bet- 
ter understood,  that  in  patients  with  tertiary  lesions  on  the 
skin,  mucous  membranes  and  bones,  tabes  is  rarely  found, 
as  well  as  the  fact  that  tabes  is  especially  frequent  after 
the  milder  attacks  of  lues  which  Fournier  describes,  and  for 
this  reason  is  the  more  dangerous.  A  further  explanation 
may  lie  in  the  fact  that  in  Turkey,  Bosnia,  and  Asia  Minor 
on  account  of  the  Mohammedan  population  alcoholism  is 
lacking.  In  these  countries  also,  as  well  as  for  the  most 
part  in  other  countries  where  tabes  is  actually  rare,  civiliza- 
tion  is  upon  a  low  level.  Finally,  as  Strumpell  has  pointed 
out,  we  know  of  no  country  where  tabes  is  of  frequent 
occurrence  and  there  is  either  no  syphilis  or  very  little 
syphilis. 

Edinger's  Exhaustion  Theory. — Edinger  produced  in  an 
experimental  way  several  years  ago  posterior  column  de- 
generations. He  found  in  rats  which  he  caused  to  work  to 
the  point  of  severe  muscular  fatigue,  along  with  traces 
of  changes  in  different  parts  of  the  spinal  cord,  changes 
in  the  posterior  roots  and  tracts  which  resembled  those 
found  in  the  posterior  column  degenerations  in  humans. 

He  also  found  when  these  rats  had  been  made  artificially 
anaemic  that  after  only  a  moderate  degree  of  muscular 
fatigue  these  degenerations  appeared.  Edinger  applied  the 
knowledge  gained  in  these  experiments  to  formulating  an 
explanation  for  the  cause  of  tabes.  He  said  in  a  system 
weakened  by  lues  the  posterior  columns  became  diseased 
through  hyperfunction  because  the  supply  of  nerve-sub- 
stance in  comparison  with  its  consumption,  on  account  of 
hyperfunction,  was  insufficient.  He  assumed  that  the  sen- 
sory columns  became  affected  for  the  reason  that  the  sen- 
sory neuron  is  less  resistant  than  the  motor. 


272  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

It  is  questionable  if  the  results  of  these  experiments  on 
animals  should  be  applied  to  human  pathology.  On  the 
other  hand,  it  is  certain  that  those  who  are  not  subjected 
to  severe  physical  labor  develop  tabes  just  as  often  as,  if 
not  oftener  than,  those  who  are. 

It  may  further  be  mentioned  that  the  artificially  pro- 
duced degenerations  of  Edinger  are  acute  in  character, 
while  the  posterior  sensory  degenerations  of  tabes  are 
chronic. 

Kron  in  134  female  patients  who  were  machine  workers, 
and  had  been  for  years,  who  furnished  the  foot-power  to 
operate  their  own  machines,  found  syphilis  in  56  per  cent., 
but  not  a  single  case  of  tabes. 

Overexertion  and  exposure  to  cold,  according  to  the 
comprehensive  and  thorough  studies  of  Erb,  may  be  stricken 
off  the  etiological  category.  After  a  consideration  of  the 
pros  and  cons  in  this  discussion  one  is  forced  to  the  con- 
clusion that  syphilis  is  by  far  the  most  important  and  fre- 
quent etiological  factor  in  the  causation  of  tabes. 

The  exact  nature,  however,  of  this  relationship  between 
the  two  diseases,  as  Erb  has  repeatedly  stated,  is  still  a  sub- 
ject for  scientific  discussion  and  investigation. 

For  the  present  at  least  Striimpell's  theory  which 
assumes  tabes  to  be  the  result  of  a  syphilitic  toxin  appears 
to  furnish  the  most  rational  working  hypothesis.  Schau- 
dinn's  discovery  of  the  Spirochcete  pallida  has  contributed 
nothing  to  the  etiology  of  tabes.  No  one  should  expect 
to  find  the  spirochaete  in  the  cord  or  spinal  fluid  of  a  tabetic 
any  more  than  he  should  expect  to  find  the  Klebs-Loeffler 
bacillus  in  nerves  which  were  affected  with  a  postdiph- 
theritic  neuritis. 

The  Wassermann  reaction  in  the  blood  and  spinal  fluid 
of  tabetics  has  given  us  another  symptom  and  further  proof 
of  the  close  relationship  existing  between  syphilis  and  tabes. 
An  absolute  proof  it  fails  to  furnish,  however,  since  the 
reaction  has  been  proven  to  be  non-specific  in  character. 

At  the  present  time  the  consensus  of  opinion  on  this 
question  may  probably  be  best  expressed  in  this  manner: 
Syphilis  is  not  exclusively  the  cause  of  tabes,  it  is  not  a 


TABES  AND  SYPHILIS  273 

sine  qua  non,  but  it  is  by  far  the  most  frequent  and  im- 
portant of  all  the  other  etiological  factors. 

Atypical  Types  of  Tabes. — Those  who  see  many  cases  of 
tabes  often  observe  cases  in  which  they  are  not  absolutely 
sure  of  the  diagnosis.  In  such  cases  it  is  doubtful  whether 
they  should  be  classified  as  abortive  cases  of  tabes,  rudimen- 
tary cases,  as  Erb  calls  them,  or  cases  of  spinal-cord  syph- 
ilis which  have  recovered.  Such  cases  acquire  through  their 
long  course  or  state  of  remission  their  characteristic  stamp, 
and  for  this  reason  are  only  of  value  to  the  observer  when 
they  have  been  under  control  for  years. 

Lightning  Pains  as  an  Isolated  Symptom. — A  number  of 
years  ago  Erb  directed  our  attention  to  these  cases.  In 
the  first  place  there  are  the  cases  which  have  suffered  pains 
in  the  lower  extremities  for  years,  that  were  extraordi- 
narily severe,  of  the  character  of  lightning  pains  coming  on 
in  attacks,  and  susceptible  to  changes  of  weather. 

A  gentleman  whom  I  have  had  the  opportunity  of  ob- 
serving for  the  past  fourteen  years  belongs  in  this  category. 
I  have  examined  him  several  times  every  year,  but  have 
never  been  able  to  demonstrate  the  slightest  anomaly  of  the 
nervous  system.  Twenty-one  years  ago  he  acquired  syphilis 
and  has  had  thorough  and  repeated  treatment.  These 
attacks  of  lightning  pains  have  remained  from  the  begin- 
ning the  only  discoverable  symptom. 

In  another  case  where  the  infection  of  syphilis  dated 
back  twenty-four  years  the  patient  suffered  severe  attacks 
of  lightning  pains,  coming  on  every  two  or  three  months 
for  a  period  of  about  eight  years.  Then  there  appeared 
rather  acutely  in  the  course  of  two  or  three  months  the 
picture  of  spinal  ataxia,  the  patellar  reflexes  disappeared, 
pupil  symptoms  developed  and  in  the  period  of  about  one 
year  the  case  presented  the  usual  picture  of  a  well-advanced 
tabes. 

Such  cases  teach  us  that  with  characteristic  subjective 
symptoms  and  negative  objective  findings,  even  though  the 
subjective  symptoms  have  continued  for  many  years,  one 
should  be  guarded  in  the  prognosis. 

Absence   of  the  Patellar  Reflexes. — More  frequent  than 

18 


274  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

these  rather  rare  cases  of  lightning  pains  are  those  cases 
in  which  the  absence  of  patellar  reflexes  and  pupil  anomalies 
show  a  diseased  process  in  the  central  nervous  system. 

It  is  possible  but  not  absolutely  certain  that  those  cases 
in  which  the  objective  symptoms  manifest  themselves  by  the 
absence  of  the  patellar  reflexes,  as  well  as  slowness  in  the 
response  of  the  pupil  reaction  to  light  and  anisocoria  or 
myosis,  are  cases  of  tabes  which  have  come  to  a  state  of 
stand-still  or  quiescence.  It  is,  however,  just  as  possible 
that  local  meningitic  or  meningomyelitic  processes  which 
have  healed  with  scar  formation  constitute  the  pathological 
foundation  for  these  clinical  symptoms. 

Isolated  Pupil  Anomalies. — It  has  already  been  stated  that 
anomalies  of  the  pupil  in  size  and  reaction  in  syphilitics 
are  often  found  and  also  often  remain  permanently  as  iso- 
lated symptoms.  The  majority  of  these  cases,  although  not 
all,  present  a  lymphocytosis  and  increase  of  the  globulin  in 
the  spinal  fluid.  As  has  been  pointed  out  in  regard  to 
paralysis  of  the  oculomotor  nerve,  so  also  it  happens  in 
isolated  loss  of  the  pupil  reaction  that  this  occurs  in  non- 
syphilitics  and  then  without  pleocytosis.  Some  of  these 
cases  complain  of  no  subjective  symptoms  whatever  and 
some  suffer  attacks  now  and  then  of  lightning  pains,  but 
otherwise  appear  to  be  entirely  normal.  One  of  these  cases 
which  I  have  known  for  ten  years  is  interesting  because  of 
the  fact  that  while  he  was  affected  in  this  manner  he  mar- 
ried and  infected  his  wife  soon  after  the  marriage.  The 
wife  now  has  a  typical  tabes. 

Finally  there  are  those  cases  which  one  cannot  with  cer- 
tainty classify  as  tabes,  in  which  the  pupils  and  patellar 
reflexes  behave  as  in  ordinary  tabes,  in  which,  moreover, 
irregular  sensory  disturbances  and  an  abnormal  frequency 
to  urination  with  dysuria  exist.  In  these  cases  an  ener- 
getic antispecific  therapy  causes  a  disappearance  of  all  the 
symptoms  except  the  pupil  and  patellar  reflex  disturbances, 
so  that  the  patients  are  free  from  any  subjective  discomfort 
and  are  not  ataxic. 

Isolated  Gastric  Crises. — It  is  known  and  has  recently  been 
called  particularly  to  our  attention,  by  Dunger,  that  gastric 


TABES  AND  SYPHILIS  275 

crises  may  be  an  early  symptom  of  tabes.  If  in  such  cases 
pupil  anomalies  or  absence  of  the  Achilles  or  patellar  re- 
flexes are  found  the  diagnosis  of  tabes  cannot  be  doubted. 

The  diagnosis  is  difficult,  however,  in  those  cases  in 
which  in  former  syphilitics  gastric  crises  appear  at  longer 
or  shorter  intervals  without  the  appearance  of  a  single 
objective  symptom  on  the  part  of  the  nervous  system. 
Every  experienced  physician  knows  that  many  cases  of 
typical  gastric  crises,  both  with  and  without  hyperacidity, 
occur  in  patients  who  have  never  had  syphilis  and  whose 
nervous  system  is  objectively  sound.  In  such  cases  stomach 
neuroses  must  be  ascribed  as  the  cause  and  a  differential 
diagnosis  is  rendered  still  more  difficult.  Under  such  con- 
ditions the  examination  of  the  spinal  fluid  and  the  absence 
or  presence  of  Phase  1,  the  test  for  globulin,  and  a  deter- 
mination of  the  number  of  lymphocytes  will  furnish  the  best 
means  of  differentiation. 

Pseudotabes  Syphilitica. — The  discussion  over  the  classifi- 
cation of  the  pathological  changes  found  in  the  posterior 
columns  in  tabes  has  been  an  extensive  one.  Some  obser- 
vers, as  Charcot,  believe  this  degeneration  is  caused  by  a 
chronic  parenchymatous  inflammation  of  the  posterior  col- 
umns ;  others,  as  Frommann  and  Friedrich,  by  a  chronic 
interstitial  inflammation  (sclerosis),  while  others,  as  Erb, 
think  that  both  forms  of  degeneration  are  present. 

The  consensus  of  opinion  among  neurologists  at  the 
present  time  is  that  tabes  is  a  primary  degenerative  process 
in  the  nerve  parenchyma  and  that  it  is  not  a  specific  syph- 
ilitic disease  of  the  cord. 

Cases  of  syphilitic  disease  of  the  cord  simulating  tabes 
were  first  recognized  by  Oppenheim  and  Eisenlohr.  In 
these  cases  the  patients  had  formerly  had  syphilis  and  pre- 
sented a  number  of  tabetic  symptoms  which  under  anti- 
specific  therapy  either  disappeared  or  were  much  im- 
proved. In  some  of  these  cases  from  the  beginning  there 
were  symptoms  which  did  not  fit  the  diagnosis  of  tabes,  in 
others  these  symptoms  appeared  later  and  made  possible 
the  correct  diagnosis. 

The  pathology  in  these  cases  showed  that  instead  of  a 


276 


SYPHILIS  AND  THE  NERVOUS  SYSTEM 


primary  tabetic  degeneration  of  the  posterior  columns, 
the  process  was  a  secondary  one  and  was  caused  by  a  spe- 
cific meningitis  which  either  ensnared  the  posterior  roots 
or  destroyed  large  transverse  areas  of  the  cord,  and  thus 
produced  secondary  degenerations  in  the  roots  and  cord. 

Eisenlohr  in  1888  reported  two  ca,ses  which  clinically 
presented  almost  typical  symptoms  of  tabes  with  an  un- 
doubted history  of  syphilis,  and  at  the  autopsy  did  not  show 
the  systematic  degeneration  of  tabes  but  disease  of  the 
posterior  columns  as  the  result  of  a  specific  meningitis. 
In  one  of  these  cases  the  involvement  of  the  posterior  col- 
umns affected  the  root-zone  in  a  segment  of  the  cord,  in  the 
other  the  entire  area  of  the  posterior  columns  was  involved, 
but  this  involvement  was  limited  to  a  few  segments  only. 


Fig.  92.  Fig.  93. 

FIGS.  92  and  93. — Case  of  Eisenlohr's. 

Numerous  cases  of  this  nature  have  since  been  reported, 
so  that  in  all  cases  in  which  the  clinical  course  deviates 
somewhat  from  a  typical  tabes,  or  in  addition  to  symptoms 
of  a  classical  tabes  cerebral  or  spinal  symptoms  appear 
which  do  not  arise  from  disease  of  the  posterior  columns, 
it  becomes  our  duty  to  ascertain  whether  or  not  the  case  is 
one  of  cerebrospinal  or  spinal  syphilis  which  simulates  a 
tabes.  In  all  such  cases  one  should  administer  without 
hesitation  antispecific  treatment. 

A  rapid  development  of  the  degeneration  in  the  pos- 
terior columns,  a  change  in  the  intensity  of  individual 
symptoms,  a  change  in  the  behavior  of  the  patellar  reflexes, 
a  complication  of  paresis  or  paralysis  of  the  extremities, 
in  which  one  extremity  is  more  affected  than  the  other, 
speaks  in  favor  of  a  pseudotabes. 


TABES  AND  SYPHILIS  277 

The  affection  of  the  optic  nerve  is  also  one  of  neuritis 
instead  of  primary  atrophy,  and  the  pupils  more  often  show 
a  complete  loss  of  their  reflexes  rather  than  that  of  the 
light  reflex  alone. 

Differential  Diagnosis  of  Spinal  Syphilis.  History. — The 
diagnosis  of  syphilis  of  the  spinal  cord  can  by  no  means 
always  be  made  with  certainty.  One  should  consider  the 
probability  of  spinal  syphilis  in  every  case  of  disease  of  the 
cord,  whether  acute  or  chronic  in  character. 

At  the  beginning  of  the  discussion  of  this  subject,  it  may 
be  stated  that  there  is  no  single  clinical  symptom  which  in 
itself  is  pathognomonic  of  luetic  disease  and  which  some 
other  non-specific  affection  cannot  cause. 

The  positive  history  of  a  specific  infection  in  the  anam- 
nesis is  naturally  important  in  the  diagnosis. 

Other  Symptoms  of  Syphilis. — The  discovery  of  other  spe- 
cific symptoms  elsewhere  on  the  body  in  a  patient  with 
spinal-cord  disease,  which,  however,  is  not  often,  is  also 
important. 

In  the  differential  diagnosis  between  tuberculosis  and 
syphilis  one  should  remember  that  not  infrequently  there 
exists  in  the  same  person  a  combination  of  both  diseases. 

The  Intensity  of  Individual  Symptoms  No  Criterion  to  Judge 
of  Local  Involvement. — Sachs  was  one  of  the  first  to  call 
attention  to  the  disproportion  existing  between  the  inten- 
sity of  individual  symptoms,  and  the  extent  of  local  in- 
volvement. 

The  Brown-Sequard  Symptom-complex. — Concerning  the 
frequency  of  the  Brown-Sequard  complete  or  incomplete 
hemiparesis  where  trauma  or  a  non-specific  tumor  can  be 
excluded  this  symptom  may  be  regarded  of  great  value  in 
the  diagnosis  of  spinal  syphilis. 

Brain  Involvement. — Involvement  of  the  brain  is  A  very 
frequent  complication.  Multiple  sclerosis  also  affects  both 
the  brain  and  spinal  cord  at  the  same  time  and  by  no  means 
always  in  the  classical  form  with  nystagmus  and  scanning 
speech.  Malignant  tumors  likewise  may  involve  simul- 
taneously both  the  brain  and  cord. 

Irritative  Symptoms. — The  irritative  symptoms  which  ap- 


278  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

pear  quite  often  in  the  prodromal  stage  of  spinal  syphilis 
can  occasionally  be  utilized  in  the  diagnosis,  but  only  when 
later  symptoms  follow.  They  are  not  characteristic  alone. 

Transient  Character  of  Symptoms. — Oppenheim  and  Sie- 
merling  have  emphasized  the  fluctuating  character  of  the 
symptoms,  especially  with  regard  to  the  patellar  reflexes. 
There  has  been  observed,  however,  in  both  extra-  and  intra- 
medullary  tumors,  tubercular  compression  myelitis,  and 
also  chronic  anaemias,  changes  in  the  behavior  of  the  patel- 
lar reflexes.  A  fluctuation  in  the  reaction  of  the  pupils 
occurs  likewise  in  brain-tumors. 

Influence  of  Therapy. — If  under  the  administration  of 
mercury  and  iodid  the  symptoms  disappear  we  may  con- 
clude in  most  instances  that  the  affection  was  of  a  luetic 
nature,  provided  that  the  symptoms  were  not  such  as  were 
capable  of  spontaneous  recovery.  There  are  specific  gum- 
matous  processes  which  very  often  prove  to  be  refractory 
to  treatment,  and  one  must  consider  that  scar  tissue  in  the 
spinal  cord,  which  pathologically  represents  a  recovery, 
may  produce  the  same  disturbance  of  function  as  an  un- 
healed  luetic  process. 

Spinal  Neurasthenia.— In  some  cases  in  the  prodromal 
stage  of  spinal  syphilis  differential  diagnostic  difficulties 
between  spinal  neurasthenia  and  spinal  syphilis  may  pre- 
sent themselves.  Neurasthenics  complain  about  pains  in 
the  back,  stiffness  of  the  back,  and  tenderness  to  both 
pressure  and  percussion.  There  is  also  often  a  sensation 
of  paraesthesias  and  heaviness  in  the  limbs  and  a  tendency 
to  become  exhausted  easily. 

If  a  history  of  syphilis  is  present  in  the  anamnesis  of  the 
patient,  often  the  further  course  of  the  disease  must  be 
awaited  in  order  to  make  a  definite  decision. 

Here  again  the  spinal  fluid  examination  and  the  absence 
or  presence  of  Phase  1  (globulin  test)  and  the  Wassermann 
reaction,  according  to  Hauptmann's  method,  should  be  re- 
garded of  great  value  in  deciding  the  diagnosis. 

Compression  Myelitis  from  Tubercular  Caries. — One  may 
only  be  permitted  to  assume  a  tubercular  compression  mye- 
litis when  disease  of  the  vertebral  column  can  be  demon- 


TABES  AND  SYPHILIS  279 

strated,  although  we  know  such  disease  may  exist  in  a  latent 
form  for  a  long  time  when  the  patient  has  tuberculosis 
elsewhere.  When  a.  specific  infection  has  existed  in  a 
patient  with  a  tubercular  inheritance  and  demonstrable 
tubercular  disease  it  may  require  a  long  period  of  observa- 
tion to  determine  the  correct  diagnosis. 

A  man  thirty- six  years  old  was  admitted  to  the  Eppen- 
dorf  Hospital  with  tuberculosis  of  the  bones  in  the  left 
hand.  After  the  bone  tuberculosis  had  healed  he  was  trans- 
ferred to  another  department  because  of  a  nephritis.  Two 
years  before  this  he  had  contracted  syphilis.  He  then  de- 
veloped the  clinical  picture  of  a  chronic  dorsal  myelitis. 
Under  inunctions  considerable  improvement  in  the  spastic 
symptoms  occurred.  The  patient,  however,  died  of  chronic 
albuminuria  and  a  secondary  cachexia.  There  had  been  no 
evidence  of  a  tuberculosis  of  the  internal  organs. 

At  the  autopsy  a  tubercular  caries  of  the  upper  dorsal 
vertebras  with  a  tubercular  peripachymeningitis  in  the  lower 
cervical  and  upper  dorsal  regions  was  found.  There  was 
also  amyloid  degeneration  of  the  kidneys,  but  no  evidence 
of  tuberculosis  of  the  internal  organs.  As  evidence  of  the 
past  syphilis  there  was  a  double  interstitial  orchitis. 

In  such  cases  in  the  spinal  fluid  both  the  globulin  and 
lymphocytes  may  show  a  considerable  increase. 

Spinal-cord  Tumors. — They  may  also  present  diagnostic 
difficulties,  as  the  following  case  illustrates : 

A  patient  in  my  department  at  Eppendorf  had  a  spastic 
paraplegia  with  a  slight  disturbance  of  the  bladder,  hypo- 
aesthesia  of  the  lower  extremities  and  lower  half  of  the 
abdomen,  and  rigidity  and  sensitiveness  of  the  vertebral 
column.  These  symptoms  began  four  years  after  a  syph- 
ilitic infection.  Antispecific  treatment  caused  no  improve- 
ment. The  radiating  girdle-pains  were  very  severe.  Decu- 
bitus  developed.  The  patient  committed  suicide  before  we 
had  the  opportunity  to  make  an  exploratory  laminectomy. 

The  postmortem  revealed  a  fibroma  the  size  of  a  walnut. 
The  growth  was  situated  on  the  dura  between  the  seventh 
and  ninth  dorsal  segments. 

We  know  at  the  present  time  that  tumors  of  the  spinal 


280  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

membranes  are  not  so  rare  and  it  becomes  our  duty  in  dif- 
ferential diagnosis  to  give  them  careful  consideration.  In 
those  cases  where  multiple  neurofibromata  are  found  under 
the  skin  the  task  is  not  so  difficult.  A  number  of  cases  of 
this  kind  have  been  reported. 

It  is  worthy  of  mention  and  evidently  not  well  known 
that  in  multiple  neurofibromatosis  the  nerve-roots  are  often 
extensively  affected. 

The  examinations  of  the  spinal  fluid  in  compressing 
spinal-cord  tumors  up  to  the  present  time  indicate  that 
lymphocytosis  can  occur  and  also  that  a  sort  of  typus  in- 
versus  may  often  be  present,  a  very  strong  globulin  reac- 
tion without  any  increase  of  lymphocytes. 

Multiple  Sclerosis. — The  differentiation  of  a  chronic  syph- 
ilitic myelitis  from  a  multiple  sclerosis  is  often  extremely 
difficult.  There  is  probably  no  differential  diagnostic  ques- 
tion in  the  sphere  of  syphilis  of  the  nervous  system  which 
occurs  so  frequently.  Both  affections  may  have  the  same 
beginning  with  motor  weakness  and  spastic  symptoms  in 
the  lower  extremities,  transient  paralyses  of  the  eye  mus- 
cles, and  temporary  disturbances  with  the  bladder.  Both 
may  exhibit  intercurrent  improvements,  both  attack  by  pref- 
erence patients  during  the  first  half  of  their  life,  and  both 
may  show  remissions  after  antispecific  treatment,  the  one 
post  hoc  and  the  other  propter  hoc.  In  many  cases  the 
determining  symptoms,  such  as  a  characteristic  nystagmus, 
optic  atrophy,  well-marked  intentional  tremor,  a  shaking 
head  or  body,  sooner  or  later  will  clarify  the  situation. 

Oppenheim  was  one  of  the  first  to  point  out  that  the 
atypical  form  of  pure  spinal  multiple  sclerosis  is  not  so 
rare  an  affection. 

Case  of  spinal  multiple  sclerosis : 

A  woman,  thirty-nine  years  old,  married  five  years, 
marriage  sterile,  ten  years  before  contracted  a  lip-chancre. 
The  initial  lesion  and  several  attacks  of  secondary  symp- 
toms were  thoroughly  treated  with  antispecific  treatment. 
Since  that  time  the  patient  had  observed  no  further  manifes- 
tations of  syphilis.  The  present  affection,  which  had  ex- 
isted eighteen  months,  began  with  paraesthesias  and  motor 


TABES  AND  SYPHILIS  281 

weakness  in  the  lower  extremities.  Gradually  a  spastic 
paralysis  developed,  which  not  only  rendered  standing  and 
walking  but  also  the  movements  of  her  limbs  in  bed  impos- 
sible. The  upper  extremities  became  slightly  paretic  and 
inside  of  one  year  developed  into  a  state  of  marked  contrac- 
ture.  Disturbances  of  sensation  appeared  in  the  form  of 
hypoaesthesia  for  touch,  temperature,  and  pain.  The  blad- 
der was  paretic.  All  the  cranial  nerves,  including  the  optic 
and  the  nerves  controlling  the  pupils,  remained  entirely 
normal.  Four  years  after  the  beginning  of  the  disease  the 
patient  died  of  severe  decubitus.  The  autopsy  showed  an 
advanced  stage  of  multiple  sclerosis  of  the  spinal  cord. 
There  were  no  evidences  of  syphilis  either  in  the  cord  or 
internal  organs. 

The  Brown-Sequard  symptom-complex  can  in  rare  cases 
occur  transiently  in  multiple  sclerosis. 

The  differential  diagnosis  has  been  made  especially  diffi- 
cult between  these  two  diseases  by  two  recently  established 
clinical  facts.  First,  because  of  sclerotic  disease  of  the 
posterior  roots,  which  Dinkier  and  Nonne  have  shown  may 
occur  in  multiple  sclerosis,  severe  radiating  pains  may 
appear  in  the  course  of  the  affection.  Second,  psychic  dis- 
turbances in  the  form  of  confusion,  maniacal  excitement, 
enfeebled  grandiose  ideas,  mental  conditions  which  have 
long  been  recognized  as  occurring  in  brain  syphilis,  may 
also  occur  in  multiple  sclerosis. 

Pleocytosis  and  the  globulin  reaction  are  not  infre- 
quently present  in  the  spinal  fluid  of  patients  with  multiple 
sclerosis.  The  Wassermann  reaction  in  the  spinal  fluid 
according  to  Hauptmann's  method  at  the  present  time  is 
undoubtedly  the  best  means  of  differentiation  between  these 
two  affections. 

Combined  Tract  Disease. — The  differentiation  between 
combined  tract  disease  and  pseudosystem  disease  lies  in 
the  fact  that  the  development  and  sequence  of  the  symptoms 
in  the  latter  is  different  from  those  in  the  pure  forms  of  a 
combined  tract  affection.  In  the  latter  the  symptoms  appear 
slowly  and  gradually,  belonging  at  first  chiefly  to  one  sys- 
tem, then  later  other  symptoms  appearing  which  show  that 


282  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

another  system  has  been  affected.  The  symptoms  are  then 
either  chronically  progressive  or  the  quiescence  period  is 
only  temporary.  An  actual  retrogression  does  not  occur. 

On  the  other  hand  in  pseudosystem  disease  symptoms 
appear  irregularly  belonging  to  one  system  or  another, 
the  regularity  in  progression  is  absent,  marked  improve- 
ments in  the  clinical  course,  and  a  partial  or  complete  disap- 
pearance of  individual  symptoms  are  often  observed.  This 
is  especially  true  in  disease  of  the  posterior  and  lateral 
columns  due  to  anaemic  processes.  The  symptoms  of  the 
disease  here  sometimes  entirely  disappear. 

Hysteria. — Hysteria  does  not  play  as  important  a  part 
in  spinal  as  it  does  in  cerebral  syphilis.  Nevertheless  now 
and  then  cases  occur  in  which  for  a  long  time,  because  of  the 
capriciousness  and  spasmodic  character  of  the  symptoms, 
the  changes  in  the  intensity  of  the  subjective  complaints, 
and  the  apparent  influence  of  suggestive  methods,  a  hys- 
terical basis  for  the  disease  may  be  assumed.  This  becomes 
more  easily  understood  when  we  remember  that  objective 
somatic  symptoms  may  be  absent  in  the  beginning  of 
organic  disease. 

Hysteria  may  occur  also  in  combination  with  lues  of  the 
nervous  system  as  it  does  with  brain-tumors  and  multiple 
sclerosis. 

Prognosis  of  Spinal  Syphilis. —  The  opinions  of  different 
authorities  vary  as  to  the  prognosis  of  spinal  syphilis. 

General. — Gebhardt  says  that  the  earlier,  more  energetic, 
and  longer  continued  the  treatment,  the  sooner  a  recovery 
can  be  attained,  and  if  one  is  able  to  make  a  diagnosis  early 
the  majority  of  the  cases  will  get  well.  Williamson  regards 
the  prognosis  of  spinal  as  better  than  that  of  cerebrospinal 
syphilis,  while  Oppenheim  holds  the  opposite  view.  It  is 
readily  understood  that  an  advanced  age,  existing  disease 
in  other  organs,  other  intoxications,  among  which  alcohol 
may  be  placed  first,  or  any  other  cachetic  condition  render 
the  prognosis  worse.  This  is  true  in  all  forms  of  syphilis 
of  the-  nervous  system. 

As  has  been  stated  in  the  chapter  on  "Syphilis  and  the 
Spinal  Cord,"  the  most  frequent  form  of  spinal  lues,  menin- 


TABES  AND  SYPHILIS  283 

gitis  and  meningomyelitis  in  a  relatively  small  transverse 
area  of  the  cord  will  produce  more  severe  disturbances  of 
function  than  upon  the  large  area  of  the  surface  of  the 
brain. 

The  same  thing  is  true  in  disease  of  the  vessels  caused 
either  directly  or  indirectly  by  syphilis.  An  obliterating 
endarteritis  in  the  cord  will  cause  naturally  a  greater  dis- 
turbance of  function  than  in  the  brain.  In  general  one  can 
say  that  in  the  cord  compensatory  substitution  will  always 
be  much  less  than  in  the  larger  brain-mass. 

Prognosis  Depends  Upon  the  Form  of  Spinal  Disease. — The 
prognosis  also  varies  with  the  form  of  spinal  syphilis.  The 
small-celled  infiltration  of  the  meninges  as  well  as  the 
gummatous  infiltrations  into  the  membranes  and  cord  are 
the  most  capable  of  regression.  If  the  clinical  picture  ex- 
hibited is  due  to  an  acute  transverse  myelitis  or  a  chronic 
myelitic  paralysis  of  this  type  the  outlook  for  recovery 
is  by  no  means  poor.  The  recovery  may  be  a  complete  one 
or  a  recovery  with  a  paretic  weakness  of  the  extremities 
and  reflex  anomalies  due  to  the  formation  of  scar  tissue 
at  the  spot  where  the  specific  tissue  had  previously  formed, 
or  the  process  may  come  to  a  state  of  quiescence  until  after 
a  longer  or  shorter  period  it  lights  up  again  in  the  same 
place  or  in  another  part  of  the  nervous  system. 

In  transverse  disease  a  lesion  in  the  dorsal  cord  is  more 
favorable  than  one  in  the  lumbar  and  sacral  cord  because 
of  the  paralysis  of  the  bladder  and  rectum  when  situated  in 
the  latter  and  the  dangers  of  decubitus.  An  involvement 
of  the  medulla  is  especially  dangerous. 

The  syphilitic  spinal  paralysis  of  Erb  because  of  its 
relatively  more  benign  nature  occupies  a  special  position  in 
the  prognosis,  since  the  pure  forms  of  this  affection  fre- 
quently regress  to  a  certain  extent  and  come  to  a  halt. 

If  the  acute  and  chronic  myelitis  depends  upon  a  necrosis 
of  the  nerve-substance,  due  to  a  shutting  off  of  the  blood 
supply,  improvement  is  out  of  the  question.  In  these  cases 
cystitis  and  decubitus  with  their  consequences  are  the  cause 
of  death. 

With  the  exception  of  the  "syphilitic  spinal  paralysis" 


284  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

the  prognosis  in  the  specific  system  affections  is  not  good 
because  these  forms  are  progressive  in  nature  and  are  unin- 
fluenced by  treatment.  They  are  not  the  direct  cause  of 
death  but  shorten  the  life  because  the  patient  becomes  weak- 
ened in  his  natural  resistance. 

Prognosis  Not  Influenced  by  Previous  Treatment. — The 
curable  forms  of  meningitis  and  meningomyelitis  show  a 
better  prognosis,  the  sooner  treatment  is  administered  and 
the  more  intelligently  and  energetically  it  is  carried  out. 
As  is  true  in  syphilis  of  the  brain  so  it  is  also  true  in 
syphilis  of  the  spinal  cord,  the  prognosis  does  not  appear  to 
be  influenced  by  previous  antispecific  therapy  during  the 
initial  and  secondary  stages  of  the  disease.  The  prognosis 
seems  to  be  equally  as  good  in  those  cases  which  never 
received  any  earlier  treatment  as  in  those  which  were 
thoroughly  treated.  When  the  nervous  system  has  become 
affected  the  influence  of  the  therapy  depends  chiefly  upon 
the  form  of  the  syphilitic  disease. 

Syphilitic  symptoms  appearing  soon  after  the  infection 
usually  have  a  better  prognosis  than  those  which  appear  a 
long  time  afterwards,  but  even  then  there  are  exceptions. 

Relapses. — When  a  spinal  paralysis  has  entirely  disap- 
peared the  patient  is  by  no  means  to  be  regarded  as  free 
from  danger.  The  possibility  of  a  relapse  is  always  threat- 
ening. 

The  old  expression  "Syphilis  does  not  die,  it  only 
sleeps"  often  renders  disappointing  our  apparently  most 
satisfactory  therapeutic  results. 


XIV 


THE  cerebrospinal  form  of  syphilis  is  by  far  more  fre- 
quent than  either  the  spinal  or  cerebral  forms  alone.  This 
is  as  one  would  naturally  expect  it  to  be,  because  the  pia, 
arachnoid,  and  dura  of  the  brain  and  cord  are  continuous 
with  one  another.  The  veins  and  arteries  of  the  cord  form 
likewise  an  unbroken  unit,  and  the  nervous  elements  bear 
also,  apart  from  the  individual  differences  of  the  ganglion- 
cells  performing  different  functions,  the  same  character. 

Either  Brain  or  Spinal  Symptoms  Frequently  Predominate.  — 
In  many  cases  one  speaks  of  brain-  syphilis  because  the 
cerebral  symptoms  predominate  to  such  an  extent  that  the 
spinal  symptoms  are  neglected;  in  other  cases  the  reverse 
occurs.  There  are  numerous  observations  in  the  literature 
where  extensive  syphilitic  changes  have  occurred  in  both 
the  brain  and  cord  that  have  not  been  revealed  in  the  clini- 
cal course.  The  following  is  a  case  which  came  under  my 
observation.  A  case  of  meningomyelitis  of  the  cervical  and 
lumbar  cord  in  the  course  of  four  months  died  of  cysto- 
pyelitis.  Repeated  examination  in  this  case  did  not  reveal 
any  brain  symptoms.  The  autopsy,  in  addition  to  the 
meningomyelitis,  showed  a  localized  gummatous  meningeal 
affection  at  the  base  of  the  pons. 

A  fact  which  experience  in  brain-tumors  has  particularly 
emphasized  is  that  the  pathological  changes  are  frequently 
far  more  intense  and  extensive  than  the  clinical  symptoms 
would  lead  us  to  suppose. 

Is  Involvement  of  the  Brain  More  Often  Latent?  —  In  gen- 
eral, a  lesion  of  the  brain  is  much  more  apt  to  escape  notice 
than  one  in  the  spinal  cord  because  of  the  larger  and  more 
numerous  indifferent  areas  in  the  brain.  A  gummatous 
nodule  in  the  brain,  a  localized  meningitis,  an  endarteritic 
or  endophlebitic  affection,  so  long  as  no  main  trunks  are 
involved,  can  escape  notice  for  a  long  time.  Frequently  this 

285 


286  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

latence  is  only  an  apparent  one.  Apparently  irrelevant 
discomforts,  as  transient  attacks  of  headache,  dizziness, 
mental  depression,  disturbance  of  sleep,  because  they  pass 
away,  are  forgotten. 

Pathology. — A  glance  at  the  pathology  of  cerebrospinal 
syphilis  shows  the  most  varied  combinations..  In  the  brain 
only  an  endarteritis  with  its  consequences  may  exist,  while 
in  the  spinal  cord  there  may  be  a  specific  meningomyelitis 
both  with  and  without  arterial  changes,  and  with  and  with- 
out gummatous  infiltrations  into  the  meninges  and  the  sub- 
stance of  the  cord.  On  the  other  hand,  the  spinal  cord 
may  present  the  well-known  arterial  and  venous  disease 
with  secondary  softening  and  sclerosis  in  combination  with 
different  specific  changes  in  the  brain.  The  disease  of  the 
brain  may  be  a  multiple  and  extensive  one,  while  the  involve- 
ment of  the  cord  is  limited  to  one  form  and  a  slight  one,  and 
vice  versa. 

Finally,  specific  disease  may  be  evenly  distributed  in 
the  brain  and  cord,  as  cases  reported  by  numerous  ob- 
servers prove. 

Cases  of  meta syphilitic  disease,  such  as  tabes  and 
paresis  in  combination  with  specific  gummatous  affections, 
which  not  infrequently  occur,  are  not  classified  here. 

The  two  most  frequent  forms  of  cerebrospinal  syphilis 
are  Heubner's  disease  of  the  blood-vessels  and  the  different 
types  of  meningitis. 

The  microscopic  examination  sometimes  reveals  the 
varied  character  of  the  meningeal  and  vessel  disease  when 
the  macroscopical  appearance  shows  only  a  gummatous 
tumor.  The  microscope  often  enables  one  to  recognize 
that  the  diseases  in  the  brain  and  spinal  cord  were  contem- 
poraneous or  that  the  lesion  in  the  brain  was  of  an  earlier 
date  than  the  one  in  the  cord. 

The  Postsyphilitic  Changes. — The  postsyphilitic  changes 
which  affect  both  brain  and  cord  represent  another  large 
category. 

The  postsyphilitic  arteriosclerosis,  with  softenings  and 
hemorrhages  which  lead  to  ascending  and  descending  de- 
generations, is  the  most  important  of  these.  Arterioscle- 


CEREBROSPINAL  FORM  OF  SYPHILIS  287 

rosis  and  tabes  frequently  occur  together.  A  postsyphilitic 
arteriosclerosis,  with  and  without  processes  of  softening, 
is  also  found  in  combination  with  cerebral  meningitis  and 
gummatous  tumors,  Eumpf  has  reported  clinically  the 
combination  of  cerebral  hemiplegia  with  tabetic  disease, 
cerebral  monoplegia  beginning  both  with  and  without  con- 
vulsions with  tabes,  the  occurrence  of  syphilitic  epilepsy 
with  tabes,  and  the  appearance  of  pupil  anomalies  and  dis- 
ease of  the  optic  nerve  with  chronic  and  subacute  specific 
myelitis. 

Reumont  has  called  attention  to  the  frequency  of  cere- 
bral apoplexy  in  combination  with  tabes,  also  the  occur- 
rence of  cerebral  hemiplegia  and  spinal  paraplegia  and  the 
coexistence  of  tabes  and  symptoms  of  basilar  meningitis. 

Fournier  has  reported  cases  in  which  transient  hemi- 
plegias  and  paraplegias  preceded  the  development  of  a  typi- 
cal tabes. 

A  case  of  cerebrospinal  meningitis  presenting  symptoms 
from  the  base  and  convexity : 

Patient  was  a  boy  eighteen  years  old.  The  syphilitic 
infection  was  two  years  old.  He  was  admitted  to  the 
hospital  because  of  severe  headache.  At  the  time  of  his 
admittance  he  was  in  a  dazed  condition,  sometimes  entirely 
awake,  sometimes  as  in  a  dream,  or  half  awake.  He  com- 
plained also  of  a  pressure  over  the  eyes.  He  had  a  double 
optic  neuritis  of  the  congestive  character,  moderate  mydri- 
asis,  and  slowness  of  the  pupil  reaction  to  light.  The  patel- 
lar  reflexes  on  both  sides  were  absent.  In  spite  of  vigorous 
antispecific  treatment  a  paresis  of  the  abducens  nerves, 
as  well  as  of  the  right  trochlear,  soon  developed.  In  the 
meantime,  however,  the  consciousness  had  become  entirely 
clear.  During  the  following  period  the  behavior  of  the 
pupils  to  light  varied.  One  day  the  left  pupil  would  react, 
and  a  few  days  later  the  reaction  to  concentrated  light 
would  be  lost.  This  variation  in  the  reaction  of  the  left 
pupil  continued  for  a  period  of  over  two  weeks,  when  the 
right  pupil;  whose  light  reaction  had  been  lost,  began  to 
show  signs  of  a  slight  reaction.  Six  weeks  later  both  pupils 
responded  promptly  to  both  light  and  accommodation.  A 


288  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

week  later,  however,  for  two  days  the  light  reaction  again 
was  lost. 

The  paresis  of  the  eye-muscles  had  disappeared  and  the 
patient  seemed  to  be  improving  nicely.  One  evening,  with- 
out any  warning,  six  weeks  after  he  came  to  the  hospital, 
he  was  seized  with  an  epileptic  convulsion,  which  began  in 
the  right  side  of  his  body  and  then  becoming  general,  con- 
sciousness was  entirely  lost.  Several  similar  attacks 
occurred  in  the  course  of  the  next  four  months,  either  severe 
like  the  first  or  more  abortive  in  character.  A  sensation  of 
tingling  appeared  in  the  right  side,  immediately  after  the 
first  attack,  but  gradually  disappeared.  At  the  time  the 
patient  was  discharged,  because  he  insisted,  the  subjective 
symptoms  consisted  in  occasional  headaches  and  attacks  of 
dizziness.  Both  pupils  reacted  both  to  light  and  accommo- 
dation, but  not  promptly,  and  the  excursion  of  the  pupils 
was  not  of  normal  extent.  There  existed  in  both  optic 
nerves  a  slight  neuritic  atrophy.  The  patellar  reflexes  were 
entirely  absent.  Other  spinal  symptoms  could  not  be 
observed. 

This  case  is  a  typical  one  with  cortical  and  basilar  symp- 
toms, in  which  the  appearance  and  disappearance  of  new 
symptoms  was  unmistakable  and  in  which  the  variable 
course  finally  terminated  in  incomplete  recovery. 

Combination  of  multiple  basilar  symptoms  with  a 
meningomyelitis  in  which  the  spinal  symptoms  remained 
while  the  cerebral  symptoms  disappeared  under  antispecific 
therapy : 

A  woman,  forty-eight  years  old,  was  infected  by  her 
husband  twelve  years  before.  The  husband  died  from  tabes. 
When  the  patient  was  admitted  to  the  hospital  she  had  a 
double  optic  neuritis  and  well-marked  specific  choroiditis. 
She  had  also  ptosis  on  the  left  side  and  on  the  right  an 
abducens  and  facial  paralysis.  In  addition  to  severe  girdle- 
pains,  there  was  a  spastic  paraplegia  inferior  of  the  Brown- 
Sequard  type.  Sensation  was  more  involved  on  the  right 
side,  motility  on  the  left.  There  were  also  sphincter  dis- 
turbances. Under  treatment  the  cerebral  symptoms  in  re- 
missions slowly  disappeared,  also  the  girdle-pains,  but  the 


CEREBROSPINAL  FORM  OF  SYPHILIS  289 

myelitic  symptoms  remained  stationary,  in  spite  of  most 
energetic  treatment,  for  a  period  of  ten  years,  when  the 
patient  died  with  symptoms  of  bronchitis  and  heart 
weakness. 

The  autopsy  showed  a  slight  chronic  thickening  of  the 
pia  on  the  base  of  the  brain,  while  in  the  dorsal  cord  a  scle- 
rosis beginning  at  the  pia  and  extending  into  the  cord  was 
found.  The  general  shrinking  in  the  cord  indicated  that 
the  process  had  probably  been  healed  for  ten  years. 

These  two  cases  may  be  regarded  as  classical  ones  of 
the  cerebrospinal  form  of  syphilis. 

There  is  scarcely  another  disease  of  the  central  nervous 
system  which  can  present  so  variable  a  course  as  that  of 
tabes.  There  is  hardly  a  cerebral  or  spinal  symptom  which 
may  not  be  manifested  by  it.  One  must  always  consider 
the  question  whether  or  not  the  existing  cerebrospinal 
symptom-complex  belongs  to  a  tabes.  This  question  is 
very  often  difficult  to  answer.  The  course  of  tabes  may  be 
such  an  unusually  variable  one.  There  are  cases  of  tabes  in 
which  the  course  is  an  acute  one,  where  in  the  period  of  a 
few  months  one  symptom  follows  another;  on  the  other 
hand,  there  are  also  cases  where  the  disease  appears  to  be 
stationary  for  many  years,  and  still  other  cases  where  it 
advances  in  periodical  relapses.  Cases  have  been  reported 
in  which  for  ten  years  the  only  objective  symptom  was  the 
loss  of  the  pupil  reaction  to  light,  and  afterwards  either 
tabes  or  paresis  developed.  In  a  case  of  my  own  the  spe- 
cific infection  dated  back  twenty  years,  a  well-marked  spinal 
myosis  twelve  years,  after  which  an  acute — almost  apo- 
plectiform — severe  tabes  developed. 

For  this  reason  the  clinical  syndrome  of  loss  of  the  light 
reaction  of  the  pupil  and  absence  of  the  patellar  reflexes 
will  always  cause  doubt  in  the  differential  diagnosis.  We 
know  that  this  combination  is  often  encountered  in  old  syph- 
ilitics  and  may  so  remain  as  isolated  symptoms  for  many 
years,  without  the  development  of  either  a  tabes  or  paresis. 
Sometimes  the  appearance  of  a  single  symptom,  such  as  be- 
ginning primary  optic  atrophy,  gastric  or  laryngeal  crises, 
or  lightning  pains,  will  clear  up  the  situation  at  once.  If 

19 


290  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

these  two  objective  symptoms  have  been  caused  by  a  syph- 
ilitic process,  one  may  expect,  almost  without  exception, 
soon  the  appearance  of  other  symptoms.  The  multiplicity 
of  symptoms  is  characteristic  of  specific  disease  of  the  cen- 
tral nervous  system,  as  well  as  their  appearance  and  disap- 
pearance and  the  variation  in  their  intensity. 

As  an  example  of  this  class  of  cases,  where  one  symptom 
follows  another  coming  both  from  the  brain  and  cord,  dis- 
appearing and  reappearing  and  varying  in  degree,  I  will 
cite  a  case  reported  by  Oppenheim.  Seven  years  after  the 
infection  spastic  paresis  of  the  lower  extremities,  with  sen- 
sory disturbances  of  the  Brown-Sequard  type,  paraesthesias, 
girdle-pains,  dysuria,  and  psychic  changes  appeared.  From 
the  eyes  there  were  diplopia,  oculomotor  paresis,  anisocoria, 
slowness  of  the  pupil  reaction  to  light,  as  well  as  ptosis  on 
the  left  side.  After  specific  treatment  a  remission  appeared. 
Then  the  symptoms  began  again  in  the  form  of  headache, 
dizziness,  vomiting,  partial  optic  atrophy,  and  paresis  of  the 
left  oculomotor  nerve ;  also  symptoms  from  the  base  of  the 
brain  manifested  themselves  in  the  form  of  dyspnoea, 
Cheyne-Stokes  respiration,  anaesthesia  in  the  areas  supplied 
by  the  fifth  nerve  on  the  one  side,  and  a  facial  paralysis. 
There  was  also  a  paresis  of  the  lower  extremities  and  one 
upper.  The  autopsy  revealed  a  gummatous  meningitis  of 
the  base  of  the  brain  and  of  the  spinal  cord,  a  gummatous 
neuritis  of  the  basilar  cranial  nerves  (optic  chiasm  and 
oculomotor,  facial  abducens  and  vagus),  an  atrophy  of  the 
roots  of  the  fifth,  as  well  as  an  arteritis  on  the  brain-base. 

In  practice  the  cases  most  frequently  encountered  are 
those  consisting  in  a  combination  of  arteriosclerosis,  post- 
syphilitic  and  true  syphilitic  disease  of  the  nervous  system. 
Such  cases,  of  course,  do  not  properly  belong  in  this 
chapter,  but  here  is  perhaps  as  suitable  a  place  to  consider 
them  as  elsewhere. 

The  following  cases  are  illustrative  of  this  combination. 

Right  endarteritic  hemiplegia,  incipient  tabes,  tertiary 
lues: 

A  widow,  forty-five  years  old,  was  taken  sick  with  a 
right-sided  hemiplegia  and  slight  motor  aphasic  disturb- 


CEREBROSPINAL  FORM  OF  SYPHILIS  291 

ances,  which  came  on  in  an  apoplectiform  attack.  Exam- 
ination disclosed  a  moderate  degree  of  arteriosclerosis  in 
the  heart  and  palpable  arteries,  also  periostitic  deposits  on 
the  anterior  surface  of  the  left  tibia,  a  double  myosis  and 
loss  of  the  pupil  reaction  to  light,  and  a  beginning  optic 
atrophy.  Under  antispecific  treatment  the  periostitic  en- 
largements disappeared  and  the  hemiparesis  markedly  im- 
proved. The  other  symptoms  remained  unchanged. 

Endarteritic  right  upper  monoplegia,  cerebral  basilar 
meningitis,  and  atypical  post  syphilitic  disease  of  the  pos- 
terior columns: 

A  man,  fifty-four  years  old,  fifteen  years  after  a  luetic 
infection,  developed  a  monoplegia  after  an  apoplectiform 
attack  of  the  right  upper  extremity.  At  the  examination, 
in  addition  to  the  hemiplegia,  there  were  found  to  be  loss 
of  the  light  reaction  in  both  pupils,  absence  of  the  patellar 
reflexes  on  both  sides,  without  any  other  tabetic  symptoms. 
Patient  also  had  an  intense  cephalalgia  and  diabetes 
incipiens. 

The  headache  and  diabetes  incipiens  disappeared  under 
antispecific  therapy.  The  other  symptoms  remained 
unchanged. 

Specific  basilar  gummatous  meningitis,  encephaloma- 
lacia,  arteriosclerosis  (hemiparesis  sinistra,  hemiparalysis 
dextra,  hemianopsia  duplex,  polydipsia  polyuria),  autopsy: 

A  fifty-four-year-old  man  ten  weeks  before  had  received 
a  blow  on  the  head  which  rendered  him  unconscious  for  a 
short  time.  During  the  next  few  days  he  complained  of 
headache  and  said  he  was  unable  to  see  as  well  as  before. 
As  a  result  of  his  poor  eyesight,  two  months  later  he  made 
a  misstep  and  sprained  his  left  ankle.  Upon  his  admit- 
tance into  the  hospital  examination  revealed  a  left  hemi- 
paresis, with  slight  exaggeration  of  the  tendon  reflexes,  a 
partial  left  homonymous  hemianopsia,  normal  eye  back- 
grounds, and  normal  condition  of  both  external  and  internal 
eye-muscles. 

A  slight  degree  of  arteriosclerosis  was  present.  He 
admitted  for  the  past  ten  years  to  have  been  a  drinker,  but 
denied  ever  having  had  syphilis.  Patient  was,  after  suit- 


292  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

able  observation,  discharged  with  the  diagnosis:  chronic 
alcoholism,  post- traumatic  arteriosclerosis,  encephalo- 
malacia. 

Three  months  later  he  was  again  admitted  to  the  hos- 
pital, after  his  disturbance  of  vision  had  increased  and 
occasional  attacks  of  dizziness,  with  unconsciousness,  had 
developed,  accompanied  at  times  with  twitching  of  the  left 
leg.  The  examination  resulted  in  the  same  findings  as 
three  months  before.  The  backgrounds  of  the  eye  still 
appeared  normal.  There  was  a  striking  polydipsia,  the 
patient  drinking  on  some  days  eight  to  ten  quarts  of  water. 
He  complained  very  much  of  headache  and  dizziness.  Nu- 
merous examinations  of  the  field  of  vision  showed  varia- 
tions in  extent  of  the  left  homonymous  hemianopsia,  also 
difference  in  the  power  of  vision  on  different  days. 

Patient  now  admitted  that  many  years  ago  he  was  in 
St.  George's  Hospital  for  venereal  disease.  By  looking  up 
the  records  it  was  ascertained  that  he  was  treated  there 
with  mercurial  inunctions  thirty  years  ago  for  chancre  and 
buboes  for  a  period  of  seven  weeks. 

The  administration  of  antispecific  therapy  was  now 
begun,  but  without  any  improvement  worth  mentioning. 

Six  months  later  the  patient  was,  for  the  third  time, 
received  into  the  hospital  because  of  the  sudden  diminution 
in  his  eyesight  three  weeks  before,  which  had  occurred 
without  any  apoplectiform  symptoms  whatever.  The  ex- 
amination showed  that  the  patient  was  able  to  see  only 
toward  the  right  side.  The  right  pupil  was  larger  than 
the  left  and  both  reacted  slowly  to  light,  and  their  excur- 
sion was  limited.  The  ophthalmoscopic  examination  now 
revealed  a  well-marked  partial  optic  atrophy.  The  left 
hemiparesis  still  remained  unchanged.  The  right  patellar 
reflex  was  absent.  The  polydipsia  and  polyuria  were  very 
much  less.  The  patient  was  again  discharged  from  the  hos- 
pital and  admitted  three  weeks  later  because  of  a  right 
hemiparesis  which  had  come  on  in  an  apoplectiform  man- 
ner. He  now  had  right  hemiparesis,  with  motor  aphasic 
disturbances,  left  hemiparesis,  almost  complete  amaurosis, 
and  still  a  moderate  degree  of  polydipsia  and  polyuria. 


CEREBROSPINAL  FORM  OF  SYPHILIS  293 

Repeated  antispecific  treatment  produced  no  change  in 
this  condition.  The  patient  became  delirious  and  suffered 
occasional  attacks  of  general  convulsions  and  attacks  of 
dyspnoea,  with  tachycardia  and  bradycardia.  Other  bul- 
bar  disturbances  were  lacking.  After  three  months  in  the 
hospital  the  patient  died  rather  unexpectedly. 

The  postmortem  showed  severe  cerebral  arteriosclerosis, 
chronic  leptomeningitis  of  the  convexity  and  base,  and  en- 
cephalomalacia  in  the  right  temporal  lobe.  Examination 
of  the  brain,  after  it  had  been  hardened  in  formaldehyde, 
revealed  just  behind  the  optic  chiasm,  on  the  brain-base, 
and  starting  from  the  pia,  an  infiltrating  gummatous  tumor 
involving  the  optic  tract,  a  part  of  the  right  internal  cap- 
sule and  the  left  optic  thalamus.  In  the  substance  of  the 
left  hemisphere,  taking  in  the  region  of  the  optic  radiations 
and  extending  into  the  internal  capsule,  was  a  compara- 
tively recent  area  of  softening,  the  size  of  a  walnut. 

The  microscopic  examination  showed  a  high  degree  of 
simple  arteriosclerosis  of  the  vessels  on  the  base  of  the 
brain  and  in  the  spinal  cord.  The  tumor  microscopically 
appeared  to  be  a  specific  gumma  with  an  exceedingly  intense 
proliferating  endarteritis  of  the  Heubner  type. 

In  the  spinal  cord,  where  macro scopically  no  change  was 
to  be  seen,  microscopically  an  incipient  tabes  was  demon- 
strated. 

This  case  teaches  us  how  a  combination  of  different 
causes  may  be  responsible  for  disease  of  the  nervous  sys- 
tem. There  was  in  the  anamnesis  syphilis,  potus,  and  a 
severe  head  injury.  It  is  interesting  because  of  the  fact 
that  even  thirty  years  after  the  infection  the  nervous  sys- 
tem was  affected  by  a  specific  gummatous  process,  also 
because  it  shows  that  true  syphilitic  processes  can  be  en- 
tirely refractory  to  antispecific  therapy,  and  further,  that 
the  assumption  of  non-specific  disease,  because  of  the  fail- 
ure of  antispecific  treatment,  is  not  free  from  objection. 
It  is  also  a  good  illustration  of  the  coexistence  of  gumma- 
tous and  specific  endarteritic  processes  along  with  post- 
syphilitic  affections  and  simple  arteriosclerosis. 

The  combination  of  a  hemiplegia  and  a  spastic  para- 


294  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

plegia  inferior,  a  triplegia,  is  a  relatively  frequent  form 
of  appearance  of  cerebrospinal  syphilis. 

Recapitulation  of  Symptoms. — In  the  recapitulation  of  the 
symptoms  of  cerebrospinal  lues  three  types  of  symptoms 
must  be  differentiated: 

.First,  those  cases  in  which  the  brain  symptoms  pre- 
dominate in  the  clinical  picture  and  the  spinal-cord  symp- 
toms are  few  and  insignificant.  These  are  cases  which  be- 
gin with  headache,  chronic  or  periodical  attacks  of  dizzi- 
ness, unilateral  or  typical  attacks  of  epilepsy,  in  which 
monoplegias  or  hemiplegias  form  the  chief  symptoms,  or  in 
which  the  multiformity  of  the  affection  manifests  itself  in 
disease  of  the  cranial  nerves.  In  addition  to  these  symp- 
toms, the  absence  of  the  patellar  reflexes,  or  bladder  dis- 
turbances, or  slight  spastic  conditions  of  the  lower  extremi- 
ties are  added. 

In  the  second  type  of  cases  the  reverse  occurs.  The  typi- 
cal picture  of  an  affection  of  the  spinal  cord  is  presented, 
which  manifests  itself  as  an  acute  or  subacute  transverse 
myelitis  which  may  be  regarded  as  the  clinical  expression 
of  a  vascular  softening  or  a  chronic  myelitis,  the  primary 
basis  of  which  is  a  disease  of  the  blood-vessels  and  very 
frequently  also  an  involvement  of  the  meninges.  In  such 
cases  sometimes  pupil  anomalies,  headache,  dizziness,  some- 
times optic  neuritis,  indicate  a  coinvolvement  of  the  brain. 

The  third  type  of  cases  are  those  in  which  both  the  brain 
and  spinal  cord  are  equally  affected.  Of  the  spinal-cord 
symptoms  those  of  irritation  may  be  the  most  prominent 
ones  for  a  long  time,  which  as  pain  and  paraesthesias  radiate 
out  from  the  back  into  the  body  and  the  extremities  and 
cause  rigidity,  and  tenderness  of  the  spinal  column  to  per- 
cussion. iSymptoms  of  root  neuritis,  which  one  is  justified 
in  diagnosing,  if  in  addition  to  the  irritative  symptoms 
atrophic  pareses  in  the  extremities  occur,  may  also  be  the 
most  prominent  symptoms  for  a  long  time.  The  motor  and 
sensory  paralyses  are  often  incomplete,  and  on  account  of 
the  more  frequent  location  of  the  lesion  in  the  dorsal  cord 
spastic  paralysis  occurs  more  often  than  flaccid.  An  early 
involvement  of  the  sphincters  takes  place  frequently. 


CEREBROSPINAL  FORM  OF  SYPHILIS  295 

The  most  frequent  of  the  cerebral  objective  symptoms 
are:  disease  of  the  optic  nerve  in  the  form  of  neuritis, 
choked  discs,  and  partial  neuritic  atrophy,  isolated  pa- 
ralysis of  eye-muscles,  ophthalmoplegia  externa  and  in- 
terna,  and  loss  of  the  pupil  reactions.  Of  the  general  symp- 
toms, headache,  dizziness,  and  epileptiform  convulsions 
occur  most  often. 

Differential  Diagnosis. — The  differential  diagnosis  of 
cerebrospinal  syphilis  is  concerned  chiefly  with  three  dis- 
eases: multiple  sclerosis,  tuberculosis,  and  multiple  sarco- 
mata and  carcinomata  of  the  leptomeninges. 

Multiple  Sclerosis. — Multiple  sclerosis,  the  same  as  cere- 
brospinal syphilis,  may  present  for  a  long  time  a  typical 
picture  of  a  spastic  dorsal  myelitis.  If  an  optic  neuritis  and 
paralysis  of  the  external  ocular  muscles  are  added  to  this, 
and  if  hemiparetic  conditions  occur,  the  clinical  syndrome 
may  for  a  period  absolutely  correspond  to  that  of  cerebro- 
spinal syphilis.  This  similarity  is  still  rendered  greater 
by  the  circumstance  that  in  multiple  sclerosis  there  is  also 
a  variation  in  the  intensity,  even  a  well-expressed  appear- 
ing and  disappearing  of  the  symptoms.  The  course  in  mul- 
tiple sclerosis,  however,  is  different.  It  is  more  chronic 
and  monotonous  and  entirely  uninfluenced  by  antispecific 
therapy.  Symptoms  which  arise  from  disease  which  in- 
volves the  individual  basilar  nerves  by  extension  do  not 
occur  in  multiple  sclerosis,  while,  on  the  other  hand,  the 
typical  scanning  speech  and  genuine  intentional  tremor,  as 
well  as  a  true  nystagmus  which  is  not  caused  by  paresis 
of  the  eye-muscles,  are  not  observed  in  syphilitic  disease 
of  the  brain. 

One  not  infrequently  encounters  cases  in  which  for  some 
time  the  differential  diagnosis  remains  in  doubt.  Such  a 
one  was  that  of  a  young  Roumanian  who  came  under  my 
observation  several  years  ago.  This  patient  presented  the 
picture  of  syphilitic  spinal  paralysis  for  almost  a  year, 
to  which  was  added  an  external  paralysis  of  the  eye-muscles, 
without  any  changes  in  the  backgrounds  of  the  eyes  or  any 
disturbances  of  speech.  The  anamnesis  in  regard  to  lues 
was  not  clear.  As  radiating  pains  in  the  extremities,  in 


296  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

addition  to  grandiose  ideas  appeared,  the  existence  of  a 
cerebrospinal  lues  seemed  to  become  more  probable,  and 
only  when,  after  an  energetic  anti  specific  treatment  with- 
out benefit,  and  when  later,  long  after  it  had  been  discon- 
tinued, the  basilar  symptoms  and  the  psychic  alterations 
spontaneously  disappeared  and  strong  contractions  devel- 
oped in  the  lower  extremities,  along  with  decubitus  and 
cystopyelitis,  was  the  diagnosis  of  multiple  sclerosis  defi- 
nitely made.  The  autopsy  confirmed  this  diagnosis. 

A  fact  brought  out  by  Dinkier,  in  1906,  with  reference 
to  multiple  sclerosis  serves  to  make  one  still  more  uncertain 
as  to  the  differential  diagnosis.  This  is  that  in  multiple 
sclerosis  girdle-like  and  radiating  pains  can  also  occur. 

The  value  of  the  four  reactions  in  the  differential  diag- 
nosis of  these  two  diseases  will  be  taken  up  in  another 
chapter. 

Differential  Diagnosis  in  Tuberculosis. — In  tuberculosis  of 
the  nervous  system,  as  in  cerebrospinal  syphilis,  the  disease 
may  predominate  in  either  the  brain  or  cord,  or  affect  both 
equally.  As  in  syphilis  so  likewise  in  tuberculosis  circum- 
scribed small  and  large  tumors  in  the  form  of  tubercles 
occur,  also  localized  and  diffuse  meningitis,  which  second- 
arily infiltrates  the  nerve  substance  or  passively  affects  it, 
and  finally,  disease  of  the  vessels  is  found  in  both,  which 
secondarily  leads  to  necrosis  of  the  nerve-substance.  Clini- 
cal reports  by  numerous  observers  demonstrate  how  great 
the  similarity  may  be  between  the  two  affections,  and,  as 
has  already  been  stated,  there  are  many  cases  in  which  only 
a  careful  review  of  all  the  factors  entering  into  the  case 
will  permit  a  diagnosis. 

Sarcoma  and  Carcinoma  of  the  Leptomeninges. — Sarco- 
matous  infiltration  may  be  distributed  over  the  entire  ner- 
vous system  and  thereby  cause  a  multiformity  in  the  clinical 
syndrome. 

Siefert  reported,  a  few  years  ago,  some  cases  which  are 
pertinent  here.  The  clinical  picture  in  these  cases  resem- 
bled very  much  that  of  an  acute  cerebrospinal  lues.  Both 
brain  and  spinal  symptoms  were  coexistent,  also  the  fluc- 
tuation of  symptoms  was  present,  especially  of  the  pupil 


CEREBROSPINAL  FORM  OF  SYPHILIS 


297 


Fio.  94. — Diffuse  sarcomatous  infiltration  of  the  pia 
mater  (middle  dorsal  region). 


and  tendon  reflexes,  and  to  such  an  extent  that  the  cases 
were  almost  all  regarded  at  first  as  hysteria.  The  course 
in  these  cases  is  usually  much  more  acute  than  is  the  case 
in  cerebrospinal  lues. 
The  result  of  lumbar 
puncture  if  one  finds  in 
the  spinal  fluid  the  tumor 
elements  will  determine 
the  diagnosis.  Lympho- 
cytosis  and  increase  of 
globulin  may  occur  in 
both  affections.  The  tu- 
mor masses  penetrate 
cone-like  into  the  cord 
and  wall  in  the  posterior 
roots. 

The  fluctuation  in  the 
intensity    of    the    symp- 
toms may  be  explained  by  the  change  in  the  fulness  of  the 
blood-vessels.    The  microscopic  examination  shows  that  the 

sarcoma-cells  press 
close  upon  the  walls  of 
the  vessels  and  the  ex- 
tra adventitial  lymph- 
spaces  fill  up  so  that 
the  lumina  of  the  ves- 
sels are  strongly  com- 
pressed. In  this  way 
temporary  retardation 
of  the  circulation  is 
caused  in  different  re- 
gions of  the  nervous 
system,  which,  how- 
ever, are  capable  of 
compensation  for  a 
long  time. 

Cysticerci. — It  has  already  been  stated  that  cysticerci 
may  cause  a  variation  in  the  intensity  of  symptoms  and  that 
in  cases  of  this  disease  the  course  may  be  a  longer  one. 


FIG.   9o. — Diffuse  sarcomatous   infiltration   of   the  pia 
mater. 


XV 

SYPHILITIC  DISEASE  OF  THE  PERIPHERAL 

NERVES 

THAT  syphilis  should  also  affect  the  peripheral  nerves 
is  easily  understood.  It  is  an  old  experience  of  the  physi- 
cian that  it  is  the  neuralgias  appearing  in  various  parts  of 
the  body  which  direct  the  attention  of  the  patient  to  his 
affliction  and  cause  him  to  seek  medical  aid. 

Secondary  Disease  of  the  Peripheral  Nerves  Caused  by 
Disease  of  the  Bones,  Lymph-glands,  Fascia,  and  Muscles. — 
The  nerve-roots,  both  sensory  and  motor,  after  their  union 
in  the  peripheral  nerve-trunk  and  the  long  course  of  the 
nerve-trunk  through  the  different  tissues  of  the  body  to  its 
termination  in  the  sensory  papillae  or  the  motor  end-plates 
in  the  muscle,  both  directly  and  indirectly  and  in  numerous 
ways  can  be  involved  in  syphilitic  disease. 

The  nerves  may,  in  their  passage  through  narrow  bony 
canals,  as  a  result  of  gummatous  periostitis  or  through 
gummatous  periosteal  nodules — as,  for  example,  in  the  in- 
tervertebral  foramina,  or  the  bony  exits  of  the  cranium — 
be  exposed  simply  to  a  compression  without  any  direct  in- 
volvement of  the  nerve-stem  in  the  local  specific  process. 

Leyden  has  stated,  in  his  work  on  diseases  of  the  spinal 
cord,  that  periostitic  nerve  compression  has  been  assumed 
more  times  clinically  than  it  has  been  demonstrated  patho- 
logically. Jiirgens  has  reported  a  case  where  gummatous 
disease  of  the  nerve-roots  existed  in  the  lumbar  region, 
together  with  severe  involvement  of  the  periosteum  of 
the  bony  environment  of  the  intervertebral  foramina. 

On  the  other  hand,  the  same  observer  has  reported  a 
large  number  of  cases  of  congenital  syphilis  in  which  he 
found  Wegner's  syphilitic  disease  of  the  bone  in  the  verte- 
bras, while  the  vertebral  periosteum  was  either  not  affected 

298 


DISEASE  OF  THE  PERIPHERAL  NERVES  299 

at  all  or  only  slightly.  After  their  exit  from  the  spinal 
canal  the  peripheral  nerves  may  be  compressed  at  any 
point  in  their  course  by  syphilitic  processes. 

There  are  naturally  points  of  predilection,  as,  for  in- 
stance, the  large  plexes  to  the  extremities  where  the  specifi- 
cally affected  lymph-glands  may  cause  pressure  on  the 
nerve-trunks  in  their  immediate  vicinity,  or  in  places  where 
the  nerve  runs  on  the  bottom  of  a  bony  groove,  as  the 
musculospiral  does,  or  where  it  is  limited  in  its  motion  by 
bands  of  fascia,  as  on  the  head  of  the  fibula. 

Primary  Disease  of  the  Peripheral  Nerves. — The  injury  to 
the  nerve-stem  is  not  always  a  passive  one,  such  as  an 
atrophy  due  to  pressure,  but  there  often  occurs  an  involve- 
ment of  the  nerve  itself,  an  infiltration  of  the  nerve-sheath, 
an  epineuritis,  which  in  the  further  advance  of  the  process 
develops  irregular  concentric  projections,  cone-shaped  in 
form,  against  the  axis-cylinder  of  the  nerve-trunk,  and  thus 
causes  a  peri-  and  endoneuritis. 

It  follows  naturally,  since  the  peri-endoneural  septum 
conducts  both  blood-  and  lymph-vessels  in  the  interior  of 
the  nerve,  that  the  vessels  should  appear  as  the  real  car- 
riers of  the  diseased  process  to  the  nerve  interior.  In  addi- 
tion to  the  chronic  traumatic  compression  atrophy  just 
referred  to,  the  blood-vessels  can  also  cause  disturbances  of 
nutrition  in  the  form  of  simple  atrophy,  necrosis,  and 
caseation. 

Syphilitic  Root  Neuritis. — This  condition  is  best  expressed 
in  the  cases  cited  at  the  end  of  the  discussion  of  the  pathol- 
ogy of  spinal  syphilis.  Kahler  was  the  first  to  describe  it 
under  the  name  of  "multiple  syphilitic  root  neuritis"  and 
it  is  analogous  to  the  already  described  forms  of  the  basilar 
multiple  root  neuritis  of  the  cranial  nerves. 

Here  likewise  the  primary  leptomeningitic  inflammation 
in  the  beginning  involves  only  passively  the  posterior,  and 
not  quite  so  frequently  also  the  anterior,  roots,  so  that 
thereby  pictures  are  presented  which  differ  in  no  way  from 
those  seen  in  the  peripheral  nerves.  If  now  the  lepto- 
meningitic induration  increases  and  there  is  joined  with  it 
a  pachymeningitic  deposit  in  such  a  manner  that  as  a  result 


300  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

of  the  adhesion  of  the  enormously  thickened  membranes, 
together  with  the  cord,  an  inseparable  mass  results  in  which 
the  nerve-roots  lie  firmly  imbedded,  one  can  understand  the 
immense  pressure  effect  which  may  be  exerted  upon  the 
extramedullary  roots. 

The  peripheral  nerves  and  the  extramedullary  nerve- 
roots  are  subject  to  specific  disease  not  only  through  pres- 
sure and  by  secondary  extension  of  the  infiltration  per 
continmtatem,  but  also  they  can  be  primarily  affected  with- 
out any,  or  at  any  rate  with  only  slight,  involvement  of  the 
meninges,  with  the  gummatous  type  of  syphilis. 

The  pathological  picture  which  is  presented  in  a  pri- 
mary root  neuritis  may  consist  of  cylindrical  cord-like  thick- 
enings or  spindle-shaped  enlargements  strung  one  after 
another,  like  pearls  on  a  string,  or,  as  in  a  case  reported 
by  Baumgarten,  grape-like  nodular  masses  in  the  course  of 
the  nerve-roots.  These  formations  may  involve  both  the 
anterior  and  posterior  roots  the  entire  length  of  the  cord, 
but  are  more  apt  to  occur  in  the  cervical  and  dorsolumbar 
regions.  The  cauda  equina  may  also  be  affected  in  the 

same  manner,  according  to  Kahane. 
In  the  majority  of  cases  of  primary 
root  neuritis  which  have  been  re- 
ported thus  far,  the  cord  membranes 
have  shown  a  greater  or  less  degree 
of  chronic  inflammatory  thickening, 
FIO.  96.— Primary  syphilitic  root  and  only  in  the  case  of  Buttersack's 

neuritis.     (Buttersack.)  ,-i  i*     1       1  • 

were  there  no  essential  changes  in 

the  meninges.  The  inner  surface  of  both  the  cerebral  and 
spinal  dura  in  its  entire  course  was  studded  with  gum- 
matous nodules,  varying  in  size  from  a  pea  to  a  lentil-seed. 

The  differential  diagnosis  between  such  cases  and  slow- 
growing  malignant  tumor  of  the  vertebra  and  meninges 
would  seem  almost  impossible. 

Peripheral  Perineuritis. — The  peripheral  perineuritis  pre- 
sents a  similar  pathology  to  the  root  neuritis.  In  fact,  there 
is  not  a  single  pathological  finding  in  the  spinal  nerves 
which  the  clinician  cannot  observe  under  his  palpating 
finger  in  the  nerves  superficially  situated.  The  nerve-trunk 


DISEASE  OF  THE  PERIPHERAL  NERVES  301 

may  be  cord-like  and  thickened,  more  or  less  sensitive,  or 
it  may  show  nodular  and  spindle-shaped  swellings  along  its 
course. 

Ehrmann  has  observed  clinically  cases  of  gummatous 
perineuritis  in  the  ulnar,  crural,  and  peroneal  nerves  which 
responded  to  antispecific  treatment. 

In  perineuritis  the  pathological  changes  consist  in  peri- 
endoneuritis  gummosa,  arteritis  of  the  vasa  nutritia,  simple 
or  degenerative  atrophy  of  the  nerve-tissue  and  a  greater 
or  less  degree  of  caseous  necrosis. 

Simple  Specific  Degenerative  Polyneuritis. — In  regard  to 
the  question  as  to  whether  there  is  a  syphilitic  simple  de- 
generative polyneuritis,  it  can  be  said  that  there  are  no 
pathological  observations  at  present  that  show  that  the 
syphilitic  poison  can  cause  in  the  nerve-trunk  a  simple 
degenerative  atrophy  or  a  parenchymatous  neuritis.  The 
clinical  fact,  also,  that  well-developed  polyneuritis  in 
patients  who  have  previously  been  syphilitic  has  responded 
to  antispecific  treatment  does  not  directly  indicate  a  poly- 
neuritis in  the  true  sense  to  have  existed.  It  is  much  more 
probable  that  specific  infiltrative  processes  are  present 
somewhere  in  the  course  of  the  affected  nerves  which  have 
escaped  observation  in  the  objective  examination. 

The  clinical  forms  of  peripheral  nerve  syphilis  manifest 
themselves  as  neuralgias,  syphilitic  neuritis  and  polyneu- 
ritis, and  root  neuritis. 

Neuralgias. — The  neuralgias  appear  relatively  frequent 
in  the  early  stages  of  specific  nervous  disease,  but  this  is 
not  always  true.  In  two  cases  reported  by  Obolensky  inter- 
costal neuralgia  appeared  in  one  case  twenty  years,  in  an- 
other eight  years,  after  the  infection. 

Neuralgia  of  the  Trifacial. — In  the  sphere  of  the  cranial 
nerves  the  trigeminal  is  especially  often  affected  in  the 
form  of  an  isolated  and  simple  neuralgia,  as  so  frequently 
is  seen  without  syphilis  after  exposure  as  so-called  idio- 
pathic  neuralgia.  The  neuralgia  of  the  fifth  in  these  cases 
is  analogous  to  the  peripheral  facial  paralysis  which  has 
been  described  as  frequently  appearing  in  the  secondary 


302  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

stage  of  syphilis.  In  such  cases  of  trigeminal  neuralgia 
the  proof  of  syphilis  in  the  history  and  its  prompt  disap- 
pearance under  antispecific  therapy  will  establish  the 
connection. 

Neuralgia  of  the  Cervical  Plexus. — Of  the  spinal  nerves 
the  branches  of  the  cervical  plexus,  the  occipitalis  major 
and  minor,  and  the  auricularis  magnus  are  more  often 
affected. 

Probably  in  no  case  of  syphilitic  neuralgia  are  the  ten- 
der spots  lacking  when  pressure  is  made  over  the  nerve  at 
the  points  where  it  conies  nearest  to  the  surface. 

The  Brachial  Plexus. — Pure  neuralgia  in  the  region  of 
the  brachial  plexus,  according  to  Rumpf,  is  rare.  However, 
I  have  seen  a  number  of  cases  which  I  considered  syphilitic 
in  character. 

Intercostal  Neuralgia. — Intercostal  neuralgia  has  fre- 
quently been  observed. 

The  following  is  a  report  of  a  case  with  several  relapses : 

A  man  who,  three  years  before  the  appearance  of  his 
neuralgia,  had  contracted  a  syphilitic  infection,  was  seized 
with  severe  pains  of  neuralgic  character  in  the  seventh  and 
eighth  intercostal  nerves  on  the  left  side.  Both  of  the 
nerves  were  along  their  course  very  sensitive  to  pressure 
and  showed  a  well-marked  hypo-anaesthesia  in  their  entire 
distribution.  No  spinal  symptoms  could  be  demonstrated. 
Under  antispecific  treatment  the  neuralgia  disappeared  in 
two  weeks.  After  six  months  the  pains  reappeared  in  the 
same  region,  and  again  responded  to  antispecific  treatment. 
After  a  year's  interval  the  neuralgia  appeared  for  the  third 
time,  but  this  time  in  the  fourth,  fifth,  and  sixth  intercostal 
on  the  opposite  side.  This  reacted  also  to  appropriate 
treatment.  No  other  evidences  of  syphilis  could  be  demon- 
strated. 

Neuralgia  in  the  Region  of  the  Lumbar  and  Sacral  Plexus. — 
Next  in  point  of  frequency  to  trigeminal  neuralgia,  accord- 
ing to  Rumpf,  are  the  neuralgias  occurring  in  the  sciatic 
nerve  of  the  sacral  plexus.  Nightly  exacerbations  of  pain 
are  said  to  be  of  especially  frequent  occurrence  in  specific 


DISEASE  OF  THE  PERIPHERAL  NERVES  303 

neuralgia  of  this  nerve.  However,  this  symptom  may  be 
said  to  have  only  a  limited  pathognomonic  significance,  as 
pain  in  all  forms  of  neuralgia  may  be  worse  at  night. 

Seeligmiiller  and  Taylor  report  the  combination  of  a 
gummatous  nodule  in  the  gluteal  region  with  a  severe  sci- 
atica of  the  same  side. 

E.  Mendel,  several  years  ago,  reported  three  cases  of 
syphilitic  sciatica.  In  the  first  case,  as  an  objective  expres- 
sion of  systemic  syphilis,  there  was  a  gummatous  tumor 
of  the  parietal  bone,  in  the  second  case  a  periostitis  of  the 
tibia,  and  in  the  third  case  the  sciatica  was  the  only  symp- 
tom. All  three  cases  responded  promptly  to  treatment.  In 
the  first  case  the  infection  dated  back  three  years,  in  the 
third  case  ten  years,  and  in  the  second  the  history  of  an 
infection  was  negative. 

Syphilitic  Neuritis  and  Polyneuritis. — Because  of  the  ab- 
sence of  a  positive  pathology  in  specific  neuritis  and  poly- 
neuritis,  the  existence  of  this  condition  has  not  been 
universally  acknowledged.  Under  these  circumstances  a 
certain  degree  of  scepticism  is  perhaps  justifiable,  but,  in 
my  opinion,  it  ought  to  yield  in  those  cases  where  a  positive 
anamnesis,  a  striking  absence  of  other  etiological  factors, 
objective  symptoms  on  palpation  of  a  perineuritis,  and  the 
prompt  response  of  antispecific  therapy,  strongly  substan- 
tiate a  specific  pathogenesis. 

The  clinical  characteristics  of  a  specific  neuritis  are 
usually  the  same  as  those  of  a  neuritis  from  other  causes. 

There  are  para?sthesias  and  sometimes  severe  attacks 
of  pain  in  the  area  of  distribution  of  the  affected  nerves. 
The  paraesthesias  are  the  most  constant  symptoms.  There 
are  almost  always  well-marked  objective  sensory  disturb- 
ances. As  has  been  stated  before,  there  are  sometimes 
found  on  palpation  along  the  course  of  the  nerve-trunk 
spindle-shaped  nodular  swellings  or  thickenings.  In  the 
motor  sphere  there  exists  a  flaccid  paresis  or  paralysis  of 
peripheral  character,  which  particularly  is  apt  to  involve 
the  musculospinal,  ulnar,  and  peroneal  nerves.  According 
to  the  severity  of  the  nerve  involvement,  there  is  a  greater 


304  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

or  less  degree  of  muscular  atrophy  in  the  affected  muscles 
with  partial  reaction  of  degeneration.  The  tendon  reflexes 
are  gone. 

It  is  rather  rare  for  a  specific  neuritis  to  be  localized  in 
a  single  nerve,  and  when  this  does  occur  the  neuritis  is 
usually  due  to  a  local  gummatous  process. 

The  following  case  is  an  example  of  this : 

A  servant  girl,  thirty-four  years  old,  was  stricken  sud- 
denly with  a  left  musculospinal  paralysis.  As  a  cause  there 
was  found  a  tumor  the  size  of  a  pigeon's  egg  in  the  muscu- 
lature of  the  left  triceps.  In  addition,  there  was  a  general 
indolent  swelling  of  the  glandular  system  and  a  slight  differ- 
ence from  the  normal  in  the  reaction  of  the  pupils.  The 
patellar  reflexes  were  absent.  There  were  no  other  demon- 
strable symptoms  of  syphilis.  No  specific  history  could  be 
obtained.  Under  antispecific  treatment  both  the  growth 
and  the  nerve  paralysis  gradually  disappeared. 

A  case  of  neuritis  of  the  right  peroneal  nerve: 

A  merchant,  fifty-seven  years  old,  whose  specific  infec- 
tion dated  back  twenty-five  years,  as  the  result  of  a  cold 
developed  pains  in  the  toes  of  the  right  foot  and  on  the 
outer  side  of  the  right  leg.  These  pains  continued  for  four 
months,  when  a  weakness  in  the  leg  appeared.  Objectively 
there  was  found  a  right  peroneal  paralysis,  a  hypoaesthesia 
on  the  outer  edge  of  the  foot,  as  well  as  a  partial  R.  D.  in 
the  peroneal  muscles.  The  trunk  of  the  nerve  behind  the 
head  of  the  fibula  was  sensitive  to  pressure.  A  mixed  treat- 
ment of  inunctions  of  mercury  and  potassium  iodid  was 
instituted,  and  after  the  tenth  inunction  the  pain  disap- 
peared, and  in  six  weeks  the  patient  was  unable  to  notice 
any  weakness  in  his  leg.  The  examination  of  the  nervous 
system  otherwise  was  negative. 

Polyneuritis. — In  specific  disease  of  the  peripheral  nerves 
a  multiple  localization  occurs  much  more  often. 

In  a  case  which  came  under  my  observation  the  infec- 
tion occurred  twenty-two  years  previously.  The  symptoms 
consisted  in  pains  in  the  shoulder,  paraBsthesias  and  pains 
of  a  neuralgic  character  in  the  entire  area,  in  a  brachial 
paresis  with  a  diminution  of  the  electrical  reaction,  in  a 


DISEASE  OF  THE  PERIPHERAL  NERVES  305 

cutaneous  hypoaesthesia  of  the  arms,  as  well  as  a  sensitive- 
ness to  pressure  and  a  slight  thickening  of  the  brachial 
plexus  in  the  subclavian  fossa. 

Ehrmann  reports  a  case  of  a  merchant,  thirty-two  years 
of  age,  who  had  contracted  lues  when  he  was  twenty-six. 
The  patient  had  a  neuritis  of  the  crural  and  a  perineuritis 
of  the  peroneal  nerve  on  the  same  side.  As  he  also  had  in- 
continence of  the  urine,  and  did  not  feel  the  feces  when  at 
stool,  and  an  anaesthetic-analgesic  zone  could  be  demon- 
strated around  the  anal  ring,  it  is  probable  also  that  there 
was  a  neuritis  of  the  pudic  nerve. 

In  1901  Cestan  published  a  report  of  thirteen  cases  of 
polyneuritis  of  specific  origin.  Two  of  these  cases  were  re- 
markable because  of  the  absence  of  both  subjective  and 
objective  disturbances  of  sensation.  For  this  reason, 
Cestan  distinguished  three  forms  of  polyneuritis  clinically. 
In  the  first  the  symptoms  are  purely  motor,  in  the  second 
they  are  both  sensory  and  motor,  and  in  the  third  pseudo- 
tabetic. 

The  justification  of  such  a  classification  cannot  be  dis- 
puted, so  long  as  the  pathology  is  lacking. 

Nevertheless  it  should  be  considered  in  many  cases — 
as,  for  example,  Cestan 's,  where  a  double  painless  musculo- 
spinal  paralysis  developed  with  B.  D.  five  weeks  after  the 
infection — whether  or  not  a  compression  of  the  anterior 
roots  by  gummatous  infiltration,  or  a  circumscribed 
meningopoliomyelitis,  or  an  arteritis  existed  instead  of  a 
polyneuritis. 

The  following  case  is  a  typical  one  of  acute  specific 
polyneuritis.  It  is  particularly  interesting  in  this  connec- 
tion, since  it  furnishes  excellent  proof  in  support  of  the 
assumption  of  a  specific  polyneuritis : 

A  girl,  twenty-two  years  old,  with  a  specific  infection 
one  year  old,  was,  four  weeks  before  her  admittance  into 
the  hospital,  taken  sick  with  parsesthesias  in  the  right  upper 
extremity,  followed  by  a  motor  weakness  in  the  muscula- 
ture of  the  shoulder  and  upper  arm.  On  examination  there 
was  found  to  be  a  paresis  in  the  right  shoulder-girdle,  right 
upper  and  lower  arm,  and  in  the  ulnar  region  of  the  right 

20 


306  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

hand.  There  was  also  a  weakness  in  the  median  and  ulnar 
region  of  the  left  hand.  There  was  a  partial  E.  D.  Sensi- 
tiveness to  pressure  existed,  and  there  were  slight  hypo- 
aesthetic  disturbances  of  sensation.  The  patellar  reflexes 
were  absent.  No  other  symptoms  could  be  demonstrated  in 
the  lower  extremities.  Later  a  complete  paralysis  gradually 
developed  in  the  lower  extremities  in  the  course  of  four 
weeks.  Occasionally,  in  addition  to  the  paraesthesias,  the 
patient  complained  of  severe  pains.  The  cranial  nerves 
were  unaffected.  The  nerve-trunks  of  the  lower  extremi- 
ties were  now  sensitive  to  pressure.  Four  weeks  later  still 
there  existed  a  paraplegia  of  all  four  extremities,  which, 
through  slight  sensory  disturbances,  tenderness  of  the 
nerves  to  pressure,  partial  B.  D.,  and  absence  of  involve- 
ment of  the  bladder  and  rectum,  left  no  doubt  as  to  the 
neuritic  nature  of  the  trouble.  On  the  neck  there  was  also 
present  leucoderma.  Other  signs  of  recent  syphilis  were 
not  discovered.  Antispecific  therapy  in  the  form  of  inunc- 
tions was  now  begun,  and  eight  days  afterwards  the  first 
evidences  of  an  improvement  in  the  paralysis  became  mani- 
fest. The  improvement  continued  uninterrupted  and  in 
four  weeks  the  patient  could  move  all  four  of  her  extremi- 
ties, and  four  weeks  later  was  able  to  stand  up  and  walk 
around.  The  neuritic  symptoms  had  all  disappeared. 

Polyneuritis  in  a  Patient  with  Probable  Congenital  Syph- 
ilis.— A  servant  girl,  twenty  years  old,  was  received  into 
my  department  at  Eppendorf  because,  six  days  previously, 
she  was  taken  sick  with  a  paresis  of  the  lower  extremities, 
accompanied  by  paraesthesias  in  legs,  feet,  and  hands.  This 
condition  increased  to  such  an  extent  in  the  course  of  three 
•days  that  the  patient  was  no  longer  able  to  stand.  She  had 
also  rather  severe  pains.  Her  hands  and  arms  were  some- 
what weaker  than  normal.  There  was  no  disturbance  of  the 
bladder  or  rectum.  Examination  revealed  a  weak,  poorly 
nourished  condition.  The  roof  of  the  nose  was  sunken  in. 
There  was  a  motor  weakness  in  the  hands  and  arms  on  both 
sides.  The  nerves  and  muscles  were  tender  to  pressure. 
The  tendon-reflexes  were  present.  In  the  lower  extremities 
the  paretic  condition  was  more  marked  and  the  tendon- 


DISEASE  OF  THE  PERIPHERAL  NERVES  307 

reflexes  were  absent.  The  electric  excitability  for  both 
currents  was  diminished,  and  there  was  a  partial  B.  D.  in 
the  median  and  ulnar  region  in  the  upper  extremity,  and  the 
peroneal  in  the  lower.  Objective  sensory  disturbances 
existed  in  the  form  of  slight  hypoassthesia. 

No  cause  for  her  disease  could  be  ascertained.  The 
hymen  of  the  patient  was  intact.  There  was  a  scar  on  the 
uvula,  one  on  the  epiglottis,  and  the  vocal  cords  were 
thickened,  which  caused  a  hoarseness.  Her  history  showed 
that  she  was  the  fifth  of  five  children,  and  the  four  who  were 
born  before  her  died  during  the  first  week  of  life.  The 
saddle-nose  dated  from  the  first  year  of  life  and  the  hoarse- 
ness from  the  tenth.  Antispecific  therapy  was  begun  and  in 
five  days  the  patient  was  able  to  stand  and  walk  a  little. 
In  four  weeks  the  recovery  was  complete. 

Multiple  Specific  Root  Neuritis. — Kahler's  classical  defini- 
tion best  describes  the  characteristic  symptoms  of  this  con- 
dition. He  says  in  a  syphilitic,  or  one  who  has  had  a  syph- 
ilitic infection,  there  may  develop  along  with  other  symp- 
toms of  cerebral  lues,  sometimes  without  such,  a  very 
stealthy  progressive  paralysis  of  the  different  cranial 
nerves,  which  one,  where  it  is  possible  as  in  the  facial 
nerve,  recognizes  as  of  peripheral  character.  One  cranial 
nerve  after  another,  in  entirely  irregular  sequence,  becomes 
affected.  In  addition  to  these  symptoms,  a  slowly  increas- 
ing neuritis  appears  in  the  region  of  the  spinal  nerves, 
accompanied  by  hypera3sthesia  or  a  girdle-sensation  and 
girdle-pain  as  a  consequence  of  progressive  disease  of  the 
posterior  spinal  nerve-roots. 

Motor  paralyses  indicate  involvement  of  the  anterior 
roots. 

In  Kahler's  case,  which  formed  the  basis  of  the  above- 
described  symptom-complex,  the  patient  was  a  young  man, 
twenty-nine  years  old,  with  an  ulcer  on  the  penis.  He  was 
stricken  three  months  after  his  infection  with  a  left-sided 
hemiplegia  and  dysarthria  without  apoplectic  symptoms. 
In  six  months'  observation  of  the  patient  there  was  added 
to  the  clinical  picture,  first  left,  then  right  facial  paralysis 
of  a  peripheral  character,  then  symptoms  of  paralysis  of 


308  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

both  oculomotor  nerves  and  vasomotor  phenomena  in  the 
skin  of  the  face.  There  was  also  a  severe  neuralgia  in  the 
region  of  the  occipital  and  some  of  the  intercostal  nerves. 

In  a  case  reported  by  Bemak  the  specific  infection  was 
twelve  years  old.  The  patient  had  complained  of  pain  for 
months  in  the  neck  and  shoulder,  which  was  followed  by 
weakness  in  the  shoulder  musculature.  In  addition  to 
exostoses  on  the  right  clavicle  and  upper  cervical  vertebrae, 
with  severe  cervicobrachial  neuralgia,  there  wa.s  a  marked 
atrophy  of  the  shoulder-girdle  and  the  flexors  of  the  upper 
arm,  paralysis  of  the  accessory,  the  long  thoracic,  paresis 
in  the  region  of  the  left  musculospiral  and  the  diaphragm, 
paralysis  of  the  vocal  cords,  absence  of  the  patellar  reflexes, 
and  E.  D.  in  the  trapezius,  sternocleidomastoid,  biceps,  and 
supinator  longus  muscles. 

This  case  furnishes  a  very  good  confirmation  of  the 
characteristics  of  root  neuritis  as  described  by  Kahler,  but 
it  also  shows  that  spinal-root  symptoms  can  be  present 
without  any  marked  involvement  of  the  cranial  nerves.  As  a 
result  of  further  pathological  observations  Kahler 's  concep- 
tion must  be  somewhat  modified  in  that  these  observations 
have  shown  it  is  not  possible  to  differentiate  between  inde- 
pendent gummatous  root  neuritis  and  the  compression  of 
the  roots  which  may  result  from  a  pachy-  and  leptomenin- 
gitic  infiltration  or  induration. 

The  observations  of  Jiirgens,  Baumgarten,  and  Wieting 
also  show  that  frequently  gummatous  processes  in  the  roots 
are  combined  with  gummatous  inflammation  of  the  spinal 
meninges. 

According  to  my  experience,  the  syphilitic  polyneuritis 
can  to  a  certain  extent  be  differentiated  from  the  root 
neuritis,  because  in  the  latter  the  pains  are  usually  of  great 
severity  and  long  duration,  they  are  also  of  a  lancinating, 
tearing  nature  and  are  increased  by  movement  of  the  verte- 
bral column,  shaking  or  jarring  of  the  body,  and  coughing 
and  sneezing.  The  pareses  and  paralyses  which  follow  the 
pain  do  not  have  the  universality  of  involvement  which  is 
found  in  the  polyneuritis,  but  resemble  more  the  spinal 
segment  type. 


DISEASE  OF  THE  PERIPHERAL  NERVES  309 

On  the  other  hand,  in  polyneuritis  paraesthesias  are  more 
frequently  observed,  the  pains  are  not  so  severe  and  stub- 
born, the  paralyses  usually  involve  the  extremities,  and 
sensitive  points  and  neuromuscular  tenderness  are  almost 
always  present. 

Oppenheim  and  Eisenlohr  have  directed  attention  to 
the  fact  that  specific  root  neuritis  may  present  symptoms 
which  resemble  tabes  and  can  be  designated  as  a  pseudo- 
tabes  syphilitica. 

Concerning  this  condition  we  have  already  spoken  in  an- 
other chapter.  One  point,  however,  should  be  emphasized 
here.  In  the  cases  described  by  Eisenlohr  it  was  remarkable 
that  in  the  beginning  the  neuralgia  was  limited  more  to  the 
regions  of  isolated  nerve-trunks  like  the  sciatic  and  it  stub- 
bornly persisted  in  this  area.  Only  in  the  further  course 
was  the  character  of  the  pain  similar  to  that  in  tabes.  It 
was  then  lightning-like,  raged  in  the  entire  extremity,  in 
the  bones,  flesh,  everywhere,  in  short,  it  radiated  out  of 
the  sphere  of  the  single  nerve-trunk  and  involved  the  whole 
limb.  There  were  also  frequently  fixed  pains  in  the  gluteal 
and  neck  regions.  In  individual  cases  the  vertebral  column 
was  constantly  sensitive  to  pressure,  and  during  the  entire 
course  of  the  disease  there  was  rigidity  of  the  neck. 

This  rachalgia,  especially  if  it  is  increased  by  passive 
and  active  movement  of  the  vertebral  column,  may  have 
a  pathognomonic  significance  ascribed  to  it,  inasmuch  as 
its  constant  presence  makes  probable  an  involvement  of 
the  pachy-  and  leptomembranes  by  a  syphilitic  process. 

In  conclusion  it  can  be  said  that  the  root  neuritis  de- 
scribed by  Kahler,  although  it  has  a  pathological  signifi- 
cance, clinically  it  can  only  claim  independent  significance 
with  the  following  supplementary  statement.  In  not  only 
primary  gummatous  processes  in  the  anterior  and  posterior 
roots  but  also  as  a  result  of  specific  pachy-  and  lepto- 
meningitic  indurations  the  appearing  compression  of  the 
same  produces  an  almost  identical  picture,  which  is  charac- 
terized by  the  early  development  of  intense  neuralgias  and 
rachalgias  with  subsequent  atrophic  paralyses. 


XVI 

HEREDITARY  SYPHILIS  AND  THE  NERVOUS 

SYSTEM 

Congenital  Syphilis  Affects  the  Nervous  System. — In  the 
discussion  of  the  influence  of  syphilis  on  the  nervous  sys- 
tem we  must  also  consider  hereditary  syphilis.  The  nervous 
system  may  be  affected  in  many  ways  by  inherited  syphilis. 

The  opinion  has  been  held  for  a  long  time,  and  Heubner 
has  recently  restated  it,  that  hereditary  lues  originates  in 
children  in  the  following  manner :  Either  the  male  or  female 
generative  cell,  or  both,  contains  the  contagion  of  syphilis 
and  this  contagion  develops  in  the  new  growing  organism 
directly  from  the  generative  cell  and  changes  it  pathologi- 
cally. 

Extra-uterine  Infection. — In  the  beginning  of  our  knowl- 
edge concerning  syphilitic  disease,  the  existence  of  a  heredi- 
tary syphilis  was  not  believed,  but  it  was  thought  in  all 
cases,  post  partum,  that  a  transmission  of  the  poison  to  the 
new-born  had  occurred.  The  wet-nurse  in  such  cases  re- 
ceived the  chief  blame. 

It  may  be  mentioned  here  that  an  especially  thorough 
examination  for  an  extra-uterine  infection  should  never  be 
omitted.  As  illustrative,  the  following  pertinent  obser- 
vation is  given : 

Convulsions  appeared  on  the  left  side  of  a  child  eleven 
months  old;  after  which  a  hemiparesis  reniained.  It  was 
ascertained  that  the  child  had  a  chancre  on  the  tongue  and 
was  nursed  by  a  syphilitic  nurse. 

The  Spirochaete  pallida. — Toe  Spiqpchate  pallida  is 
found  regularly  in  large  numb^s  in  the  nervous  system 
of  the  hereditary  syphilitic  fostuaf  as  a  part  of  the  general 
invasion  of  the  fetal  tissues.  Since  the  proof  of  the  spiro- 
chaete  in  specific  nervous  diseases  in  the  adult  is  very  diffi- 

310 


HEREDITARY  SYPHILIS  311 

cult — and,  up  to  the  present  time,  except  in  gummata,  they 
have  not  been  found — it  should  be  pointed  out  here  that 
their  presence  in  the  nervous  tissues  in  the  fcetus  is  only 
an  expression  of  their  general  invasion  of  the  fetal 
organism. 

Fundamentals  in  Hereditary  Lues. — The  most  important 
points  to  be  remembered  in  regard  to  hereditary  syphilis, 
and  which  stand  at  the  present  time  undisputed,  may  be 
summed  up  under  four  headings : 

1.  In  by  far  the  greatest  number  of  cases  syphilis  is 
transmitted  by  the  husband  to  the  wife ;  that  is,  hereditary 
syphilis  of  the  child  usually  can  be  attributed  to  the  father. 

2.  The  wife  can  be  infected  by  her  husband  either  before 
the  conception  or  at  the  time — usually  before.     In  either 
case  the  syphilis  of  the  child  is  contracted  from  both  father 
and  mother. 

3.  A  wife  may  conceive  from  a  syphilitic  husband,  with- 
out herself  becoming  infected.     The  syphilis  of  the  fcetus 
then  is  received  from  the  father.  The  mother  may  become 
infected  secondarily  from  the  syphilitic  fcetus.     However, 
this  does  not  necessarily  follow.    The  influence  of  the  luetic 
fcetus  on  the  mother  is  shown  by  the  circumstance  that  if 
she  then  remains  free  from  any  symptoms  of  syphilis,  she 
becomes  immune  to  specific  infection. 

4.  It  can  also  happen  that  both  father  and  mother,  at 
the  time  of  conception,  are  free  from  syphilis,  but  later, 
during  pregnancy,  the  mother  becomes  infected.    In  such 
cases  the  maternal  and  fetal  placenta  must  be  infected. 

From  these  statements  one  recognizes  how  complicated 
the  relations  may  be,  and  how  difficult  or  impossible  the 
anamnestic  proof  of  syphilis  with  reference  to  hereditary 
syphilis  often  is. 

The  Attenuation  of  the  Specific  Virus. — The  well-known 
teaching  of  the  gradual  lessening  of  the  syphilitic  virus 
which  is  shown  in  abortion,  the  premature  birth  of  dead 
children,  premature  birth  of  living  syphilitic  children,  the 
birth  of  full-term  children  which  immediately  show  specific 
symptoms,  or  later  develop  them,  and  the  later  birth  of 
healthy  children  who  remain  healthy,  has  many  exceptions. 


312  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

Werner,  in  Engel  Reimer's  clinic  at  the  Hamburg  Gen- 
eral Hospital,  has  recently  shown  that  not  infrequently  a 
healthy  child  may  be  born  in  between  two  syphilitic  ones. 
This  confirms  Fournier 's  teaching  that  with  a  history  of 
lues  in  the  parents  the  birth  of  a  healthy  child  is  no  guaran- 
tee for  the  future. 

Severity  of  Parental  Syphilis  Does  Not  Always  Correspond 
to  the  Degree  of  Congenital  Lues. — The  severity  of  the  paren- 
tal syphilis  does  not  always  stand  in  direct  proportion  to 
the  degree  of  hereditary  syphilis.  The  severity  of  the 
parental  syphilis  is  without  influence  on  the  frequency  of 
hereditary  syphilis.  This  is  true  for  hereditary  syphilis  in 
general,  as  well  as  for  the  acquired  and  hereditary  syphilis 
in  particular  developing  in  the  nervous  system. 

With  reference  to  the  acquired  syphilis,  a  case  reported 
by  Gilles  de  la  Tourette  and  Fournier  furnishes  a  good 
illustration.  In  this  case  the  husband  had  an  exceedingly 
mild  type.  In  the  wife  an  unusually  malignant  nervous 
syphilis  developed  in  the  brain  and  spinal  cord. 

The  literature  shows  that  in  many  severe  cases  of  hered- 
itary syphilis  the  symptoms  of  specific  disease  in  the 
parents  were  so  mild  that  they  had  not  been  observed. 
Consequently,  the  anamnestic  proof  of  syphilis  could  not  be 
obtained. 

Syphilis  of  the  Parents  May  Have  Occurred  a  Long  Time 

Back. — It  is  important  to  know  that  the  inheritance  of 
syphilis  may  take  place  although  the  parental  infection  has 
occurred  years  before.  Fournier  has  reported  a  case  in 
which  the  interval  was  fifteen  years,  Molenes  one  in  which 
the  interval  was  twenty- two  years. 

Frequency  of  Involvement  of  the  Various  Internal  Organs 
in  Hereditary  Syphilis. — The  most  frequent  autopsy  finding 
in  hereditary  syphilitic  children,  who  have  manifested  the 
usual  picture  of  a  hereditary  syphilitic  exanthema  and 
coryza,  is  that  of  marasmus.  All  the  organs  of  the  thorax 
and  abdomen  can,  however,  become  specifically  affected. 
According  to  Heubner,  the  following  is  the  order  in  point 
of  frequency:  bones,  liver,  lungs,  spleen,  alimentary  tract, 


HEREDITARY  SYPHILIS  313 

heart,  blood-vessels,  and  nervous  system.  Rumpf  gives  the 
percentage  of  nervous  involvement  as  13  per  cent,  in  heredi- 
tary syphilis. 

Late  Hereditary  Syphilis. — In  hereditary  syphilis  not  in- 
frequently years  may  elapse  before  tertiary  lesions  of  the 
skin  and  bones,  as  well  as  the  internal  organs,  including 
the  nervous  system,  appear.  One  speaks  then  of  syphilis 
hereditaria  tarda.  Various  opinions  are  held  as  to  whether 
these  tertiary  symptoms  can  appear  in  children  who  after 
their  birth  have  never  shown  any  evidences  of  syphilis. 
The  chief  champions  of  this  possibility  are  Fournier,  Eabl, 
and  Diihring.  Naturally  the  objection  can  be  made  that  one 
can  never  be  certain  that  secondary  symptoms  have  not 
been  present  intra  vitam,  and  have  thus  escaped  obser- 
vation. 

Time  of  Appearance  of  the  Late  Symptoms. — According  to 
Zeissl,  Anganeur,  and  Wolff,  these  late  symptoms  are  the 
most  apt  to  occur  during  the  age  of  puberty.  Fournier, 
from  212  cases  partly  observed  by  himself  and  partly  col- 
lected from  the  literature,  places  wide  limits  for  the  appear- 
ance of  these  symptoms,  from  the  third  year  up  to  the 
twenty-eighth  year,  with  the  maximum  of  frequency  around 
the  twelfth  year.  Lepine  and  Charcot  report  a  case  of  late 
hereditary  lues  with  brain  symptoms  in  the  thirty-second 
year  of  age. 

At  any  rate,  it  is  certain  that  the  infant,  in  a  small  pro- 
portion of  the  cases,  can  remain  free  from  relapses  for  a 
long  time,  and  that,  with  the  relapses  in  the  skin  and  mucous 
membranes,  often  gummatous  processes  appear  in  the  inter- 
nal organs  in  the  above-stated  frequency. 

The  same  granulation  tissue  is  found  in  the  internal 
organs  as  is  found  in  adults,  only  it  is  worthy  of  mention 
that  there  seems  to  be  more  inclination  to  diffuse  infiltra- 
tion than  to  circumscribed  tumor  formation. 

Symptoms  of  Hereditary  Syphilis  Apart  from  the  Nervous 
System. — The  manifestations  of  syphilis,  or  evidences 
thereof,  often  suggest  the  specific  nature  of  nervous  disturb- 
ances in  children.  An  osteochondritis  which  was  first  de- 


314  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

scribed  by  Wegner,  and  consists  of  a  disturbance  of  the 
ossification  in  the  cartilage  zone  on  the  epiphyseal  border 
of  the  long  bones,  which  leads  to  a  separation  of  the  epiph- 
ysis  because  of  the  formation  of  syphilitic  granulation 
tissue,  should  be  mentioned.  Also  coryza,  and  a  macular, 
papular,  or  pustular  specific  exanthema,  a  scaling  of  the 
epidermis  on  the  palmar  surfaces  of  the  hands  and  plantar 
surfaces  of  the  feet,  onyxis,  the  deformity  of  the  teeth  de- 
scribed by  Hutchinson,  rhagades,  and  nervous  deafness  are 
among  the  symptoms. 

Eye  Symptoms. — Excluding  the  optic  nerve  and  the  ex- 
ternal and  internal  eye-muscles,  an  examination  of  the 
eyes  will  still  furnish  much  evidence  for  the  diagnosis  of 
hereditary  syphilis.  A  careful  examination  of  the  eyes  in 
suspicious  cases  is  absolutely  necessary.  In  the  choroid 
one  should  look  for  pigment  deposits  and  choroiditic  spots 
which  have  been  left  after  a  choroiditis;  in  the  retina,  for 
inflammatory  processes  with  and  without  involvement  of 
the  choroid;  in  the  lens,  for  opacities,  usually  dust-like  in 
character.  The  iris  should  be  examined  for  inflammatory 
or  gummatous  disease,  as  well  as  synechias;  the  cornea, 
for  evidences  of  an  existing  or  past  interstitial  keratitis. 
Diseases  of  the  tear-duct  and  sac  due  to  hereditary  syph- 
ilis, in  the  form  of  dacryocystitis-scars  and  periostitis  of 
the  walls,  also  occur. 

Pathology  of  the  Nervous  System  in  Hereditary  Syphilis. — 
The  pathology  of  congenital  lues  of  the  nervous  system 
must  also  be  classified  into  genuine  specific  syphilitic 
changes  and  non-specific  syphilitic  changes. 

With  reference  to  the  non-specific  pathology,  Jarisch 
found  in  children  who  in  infancy  had  an  exanthema  and 
died  without  manifesting  any  particular  nervous  symptoms 
a  decrease  in  the  number  of  ganglion-cells,  a  change  in  the 
protoplasm  of  the  cells,  and  vitreous  masses  around  the 
vessels  in  the  central  nervous  system.  These  findings  are, 
however,  of  too  general  a  nature  to  be  acknowledged  as  fun- 
damental. Jarisch  considers  them  as  an  evidence  of  a 
diminution  in  the  general  resistance  of  the  nervous  system 
due  to  the  influence  of  congenital  syphilis. 


HEREDITARY  SYPHILIS  315 

Sibelius  holds  a  similar  opinion.  This  observer  found 
in  his  examination  of  the  central  nervous  system  in  heredi- 
tary lues  in  the  spinal  ganglia,  groups  of  partially  abnormal 
ganglion-cells  which  were  probably  due  to  delayed  or  ab- 
normal development.  In  the  very  severe  cases  of  congenital 
lues  these  colonies  or  groups  could  be  frequently  demon- 
strated, and  in  large  numbers.  iSibelius  regards  the  occur- 
rence of  these  colonies  with  marked  atypical  ganglion-cells 
as  the  result  of  restricted  development  produced  by  the 
syphilitic  toxin. 

Defective  Development. — The  severe  forms  of  defective 
or  restricted  development  of  the  central  nervous  system  are 
very  important,  and  to  a  certain  extent  characteristic  of 
congenital  lues.  Since  the  harmful  influence  of  syphilis 
transmitted  from  the  parents  to  the  foetus  encounters  first 
the  developing  central  nervous  system,  this  is  not  infre- 
quently retarded  in  its  harmonious  development  and  there 
result  extensive  deformities  or  malformations  which  are 
not  compatible  with  the  extra-uterine  life  of  the  fo3tus. 

Ilberg  described  the  central  nervous  system  of  a  luetic 
child,  six  days  old,  in  whom  both  the  liver  and  spleen  showed 
specific  syphilitic  changes.  In  this  case  the  entire  medul- 
lary substance  of  both  hemispheres  of  the  cerebrum,  the 
corpus  callosum,  the  anterior  commissure,  the  fornix,  and 
the  corpora  mammillaria  were  lacking.  There  were  also 
lacking  the  entire  pyramidal  tracts  as  far  as  the  spinal 
cord,  asymmetry  of  the  cerebellar  hemispheres,  and  devel- 
opmental deficiencies  in  the  nerve-fibres  in  the  optic  tract. 

It  should  be  stated  here  that  developmental  malforma- 
tions of  all  kinds  may  be  caused  by  other  influences  than 
parental  syphilis,  as  injuries  and  chronic  intoxications. 
Congenital  lues,  however,  in  the  causation  of  malformations 
of  the  brain  and  spinal  cord,  plays  a  very  important  part. 

Hydrocephalus. — A  comparatively  frequent  and  early- 
recognized  malformation  of  the  brain  in  hereditary  syphilis 
is  hydrocephalus.  Mendel  has  published  a  resume.  He 
states  that  Haase,  in  1828,  reported  the  case  of  a  woman 
who  was  infected  by  her  husband.  This  woman  gave  birth 
to  three  premature  still-born  children,  and  then  a  full-term 


316  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

child  which  died  in  its  seventeenth,  month  with  hydro- 
cephalus. 

Eosen  published,  in  1862,  a  number  of  cases  of  congeni- 
tally  syphilitic  children  who  died  in  the  first  years  of  life 
with  hydrocephalus. 

Many  such  cases  have  been  reported,  in  which,  in  addi- 
tion to  a  greater  or  less  degree  of  hydrocephalus,  there  was 
also  specific  disease  of  the  brain. 

In  many  cases  a  syphilitic  arteritis  is  probably  the  cause 
of  the  hydrocephalus,  and  primarily  also  the  cause  of  the 
congenital  malformations  produced  by  the  hydrocephalus. 
The  vessel  disease  promotes  the  exudation  of  lymphatic 
fluid  in  early  intra-uterine  life,  which  causes  a  watery 
accumulation  in  the  brain-vesicles  and  the  central  canal. 

Heubner  saw  as  the  cause  of  a  hydrocephalus  in  a  con- 
genitally  syphilitic  child  a  well-developed  pachymeningitis 
hsemorrhagica. 

According  to  Biedert-Vogel  congenital  chronic  hydro- 
cephalus, in  many  cases,  is  due  to  a  syphilitic  induration 
of  the  brain-meninges  and  displacement  of  the  vessels. 

Hochsinger,  several  years  ago,  made  an  exhaustive  study 
of  specific  hydrocephalus  in  infancy  from  35  cases.  In 
34  of  the  35  cases,  in  addition  to  the  hydrocephalus,  syph- 
ilitic symptoms  in  the  form  of  coryza,  exanthema,  bone, 
spleen,  and  liver  affections  were  present.  The  beginning 
of  the  hydrocephalus  occurred  in  the  majority  of  the  cases 
between  the  third  and  eleventh  months.  In  six  cases  it  be- 
gan during  the  fetal  life.  In  11  of  the  cases  there  were  no 
nervous  symptoms.  The  nervous  symptoms,  when  present, 
were  manifested  in  restlessness,  insomnia,  chronic  vomiting, 
contractures,  and  convulsions,  idiocy,  nystagmus,  and  in- 
crease of  the  tendon-reflexes.  In  a  few  of  the  cases  the 
clinical  picture  resembled  tubercular  meningitis  very  much. 

Audeont  and  Haller  report  three  cases  of  specific  hydro- 
cephalus in  hereditary  lues  which  disappeared  under  anti- 
specific  treatment. 

Concerning  the  frequency  of  hereditary  lues  as  the  basis 
of  infantile  cerebral  palsy  the  opinions  of  different  obser- 
vers vary.  Fournier  was  able  almost  always  to  demonstrate 


HEREDITARY  SYPHILIS  317 

syphilis  in  the  anamnesis  of  the  parents  or  other  antece- 
dents of  the  affected  children.  He  and  Gilles  de  la  Tourette 
have  described  cases  of  Little's  disease  (congenital  spastic 
paralysis)  as  occurring  in  hereditary  lues. 

Heubner,  Erlenmeyer,  and  Franke  also  consider  that  a 
relationship  between  infantile  cerebral  paralysis  and  con- 
genital lues  frequently  exists.  It  seems  to  me,  however, 
that  in  the  true  infantile  encephalitis,  syphilis,  as  an  etio- 
logical  factor,  does  not  play  a  very  important  part.  Oppen- 
heim  holds  the  same  opinion.  Sachs,  who  has  had  an  espe- 
cially large  experience  in  the  encephalitic  paralyses  of 
childhood,  in  200  cases  was  able  to  demonstrate  congenital 
lues  in  only  two.  The  most  important  etiological  factors 
in  the  causation  of  this  disease  appear  to  be  birth  injuries 
and  acute  infectious  diseases. 

Brain  Apoplexy. — Brain  apoplexies  are  likewise  of  rare 
occurrence  in  congenital  lues.  In  spite  of  this,  however, 
numerous  cases  have  been  reported  in  the  literature.  The 
case  reported  by  Growers  is  perhaps  the  best  known.  This 
was  of  a  boy,  twelve  years  old,  with  congenital  lues,  who 
died  suddenly  of  a  brain  apoplexy  which  ruptured  into  the 
ventricle.  The  autopsy  revealed  specific  disease  of  verte- 
bral and  deep  cerebral  arteries  as  the  cause  of  the  hemor- 
rhage. 

Disease  of  the  Blood-vessels. — Heubner 's  arteritis  is  also 
found  in  congenital  lues. 

The  endo-,  meso-,  and  periarteritis  may  represent  the 
only  pathological  change,  or  it  occurs,  as  in  the  majority 
of  the  cases,  along  with  pachymeningitis,  gummatous  proc- 
esses, and  diffuse  sclerosis. 

Disease  of  the  Meninges. — A  pachymeningitis  hemor- 
rhagica  interna  has  been  reported  by  Heubner,  Spiller, 
Waldeyer,  and  Kobner.  Hemorrhages  between  the  pia  and 
the  brain-cortex  and  in  the  ventricles  have  been  described 
by  Virchow.  Gnopf  has  reported  a  case  of  gummata  in  the 
meninges  in  a  child  thirteen  weeks  old,  which  evidently 
originated  intra-uterine. 

True  Specific  Nervous  Disease  in  Congenital  Lues  in  Com- 
bination.— The  genuine  syphilitic  diseases  of  the  nervous 


318  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

system  in  congenital  lues  are  found  almost  always  in  com- 
bination. When  syphilis  appears  in  congenital  lues  later  in 
life  the  processes  do  not  then  differ  from  those  found  in  the 
adult,  where  the  lues  has  been  acquired.  We  find  here  also 
lepto-  and  pachymeningitic  processes,  miliary  gummata, 
and  large  gummatous  tumors,  Heubner's  endarteritis,  with 
secondary  softenings,  and  neuritis  gummosa  side  by  side. 

The  most  frequent  findings  of  these  various  processes 
are  likewise  the  arterial  and  gummatous  affections.  The 
latter  condition  occurs  most  often  in  the  form  of  a  diffuse 
infiltrating  gummatous  meningitis,  or  meningoencephalitis 
and  meningomyelitis.  At  the  same  time  isolated  multiple 
gummata  are  of  frequent  occurrence. 

The  following  two  cases  are  notable  ones  of  hereditary 
lues  of  the  nervous  system : 

Case  reported  by  Siemerling:  syphilitic  neuritis  of  the 
optic  and  motor  oculi  nerves,  circumscribed  and  diffuse 
gummatous  infiltration  of  the  dura,  arachnoid,  and  pia 
mater,  meningoencephalitis  gummosa,  syphilitic  arteritis, 
specific  gummatous  infiltration  of  the  spinal-cord  mem- 
brane, and  sclerotic  changes  in  the  posterior  columns. 

Siemerling 's  case  illustrated  the  multiplicity  of  the  find- 
ings. The  nervous  disease  appeared  in  the  sixteenth  year 
of  age. 

Pick  found  in  a  child  fourteen  months  old  gummata  in 
the  frontal  lobes  and  obliterating  endarteritis  of  the  cere- 
bral arteries. 

Isolated  Spinal-cord  Syphilis  Probably  Does  Not  Occur  in 
Congenital  Lues. — I  have  never  seen  in  my  own  experience, 
nor  have  I  ever  found  in  the  literature,  a  case  reported  of 
pure  spinal  lues  in  congenital  syphilis.  The  spinal  cord 
usually  becomes  involved  in  the  form  of  disease  of  the 
arteries  and  veins,  circumscribed  and  diffuse  infiltrating 
gummatous  tumors,  meningitis,  and  root  neuritis. 

Summary  of  the  Pathological  Changes. — All  the  changes 
in  the  blood-vessels,  the  meninges,  and  in  the  nerve-sub- 
stance itself,  which  have  been  observed  in  acquired  lues, 
occur  also  in  congenital  lues.  Multiple  parts  of  the  ner- 
vous system  are  more  frequently  affected  in  congenital 


HEREDITARY  SYPHILIS  319 

lues  than  in  acquired,  and  the  various  types  of  brain  syph- 
ilis, meningitis,  gummatous  formation,  and  arteritis  are 
found  often  in  combination.  Scleroses  side  by  side  with 
genuine  specific  processes  also  occur,  as  well  as  postsyph- 
ilitic  systematic  degenerations  analogous  to  those  in 
acquired  lues. 

Clinical  Symptoms. — The  influence  of  syphilis  on  the  ner- 
vous system  in  childhood  shows  itself  more  clearly  than 
later  in  life,  because  in  the  child  the  presence  of  other 
etiological  harmful  factors,  as  alcohol,  trauma,  physical 
overexertion,  and  psychic  shock,  is  lacking.  On  the  other 
hand,  early  involvement  of  the  central  nervous  system  is 
particularly  injurious  because  it  is  attacked  during  its 
developmental  period. 

General  Debility  or  Lack  of  Vitality. — The  children  of 
syphilitic  parents  very  often  show  clinically  defective  vital- 
ity and  general  weakness. 

Werner  has  shown  from  167  children  in  Engel  Beimer's 
clinic,  whose  mothers  were  syphilitic,  that  75  per  cent.  died. 
In  the  children  who  were  manifestly  luetic  63.5  per  cent, 
died  within  the  first  year,  in  those  who  were  not,  50  per  cent. 
It  appears,  therefore,  from  Werner 's  statistics,  that  a  child 
of  luetic  parents,  although  it  may  be  apparently  free  from 
congenital  lues,  even  though  it  possesses  strength  enough 
to  develop  to  a  certain  extent,  nevertheless,  in  comparison 
to  the  offspring  of  healthy  parents,  has  suffered  greatly  in 
its  resistance.  Objectively  this  diminution  in  vital  resist- 
ance is  shown  in  the  digestion,  diarrhoeas,  disposition  to 
infectious  diseases,  and  anomalies  of  development. 

It  is  not  generally  known  that  otherwise  healthy  chil- 
dren, whose  fathers  were  syphilitic,  show  a  striking  lack  of 
resistance  to  infectious  diseases. 

This  general  state  of  debility  not  infrequently  manifests 
itself  predominately  in  the  nervous  system.  Such  children 
are  inclined  to  epilepsy  or  infantile  convulsions. 

Nervous  Form  of  Rickets. — Rickets  appears  in  them  in 
the  nervous  form;  that  is,  the  children  from  slight  causes 
are  liable  to  develop  eclamptic  convulsions.  Heubner  ex- 
plains that  such  cases  are  due  to  the  indirect  consequences 


320  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

of  syphilis,  since  after  their  death  one  may  search  in  vain 
through  the  entire  central  nervous  system  without  discover- 
ing any  visible  cause. 

According  to  Schuster,  eclampsia  often  occurs  in  infants 
who  come  from  parents  with  latent  syphilis.  Meningitic 
symptoms,  appearing  in  the  form  of  contractures  of  the 
musculature  of  the  neck  and  extremities,  somnolence,  and 
coma,  also  severe  glottis  spasm,  may  appear  in  children 
with  congenital  lues  during  the  first  year  of  life,  and  later 
cause  death,  and  at  the  autopsy  remain  unexplained. 

Not  infrequently  an  old  specific  infection  of  the  father, 
which  has  lingered  for  years,  shows  itself  in  the  child  simply 
in  the  form  of  an  irritable  weakened  nervous  system. 

Simple  Nervousness. — I  saw  this  condition  particularly 
well  expressed  in  a  child  five  years  old.  The  father  of  the 
child  had  been  infected  with  lues  six  years  before  the  birth 
of  this  child.  Two  years  before  its  birth  he  had  been 
treated  for  a  specific  iritis.  The  mother  was  not  infected. 
Neither  parent  was  nervous,  and  the  subsequent  offspring 
did  not  show  any  neuropathies.  This  child  from  the  very 
beginning  was  easily  excited.  Later  it  suffered  much  with 
headache,  abnormalities  in  disposition,  stubborn  insomnia, 
and  irregular  appetite.  A  dietetic  regimen  combined  with 
tonic  measures  was  of  no  benefit.  The  child  was  brought 
to  me  and  thoroughly  examined  without  finding  any  mani- 
fest symptoms  of  lues.  However,  as  I  had  previously  diag- 
nosed the  iritis  of  the  father,  I  prescribed  large  doses  of 
potassium  iodid  for  the  child,  with  the  result  that  the  sleep 
returned,  the  abnormal  irritability  was  lost,  and  the  little 
patient  improved  in  every  way.  At  the  present  time,  after 
twelve  years,  this  child  is  a  normal,  healthy  girl. 

I  regard  these  cases  as  very  important,  and  since  one 
can  scarcely  find  anything  concerning  them  in  the  literature, 
I  will  briefly  report  two  more. 

One  case  was  that  of  a  boy,  four  years  old,  who  was 
very  intelligent,  rather  prematurely  developed,  without  any 
signs  of  rachitis,  degeneration,  or  syphilis.  He  was  very 
restless  in  his  sleep,  had  periods  of  depression,  and  abnor- 
mally frequent  erections.  The  father  had  married  five  years 


HEREDITARY  SYPHILIS  321 

before  and  ten  years  before  had  syphilis.  He  had  taken  his 
last  antispecific  treatment  just  before  his  marriage.  Fur- 
ther observation  of  the  child  in  the  hospital  did  not  reveal 
anything  new.  Sedative  and  constructive  treatment  did  not 
produce  any  improvement.  Then  I  began  with  an  energetic 
administration  of  potassium  iodid.  From  this  time  on  a 
striking  improvement  took  place,  which  lasted,  under  the 
continuation  of  the  medication,  for  about  a  year,  when  I 
lost  sight  of  the  boy. 

In  another  case  of  a  little  girl,  four  years  of  age,  whose 
mother  was  healthy,  the  father  had  contracted  syphilis  eight 
years  before,  and  was  thoroughly  treated  at  that  time. 
This  child  was  also  easily  excited  and  prematurely  devel- 
oped. She  kept  the  family  in  terror  because  of  her  mental 
and  physical  unrest,  and  also  sleeplessness.  In  this  case 
potassium  iodid  in  large  doses  was  likewise  very  efficient. 

These  cases  show  that  a  genuine  irritability,  psychic 
unrest,  and  particularly  disturbances  of  sleep  in  children 
may  be  an  expression  of  inferior  nervous  strength  due  to  a 
previous  syphilis  in  the  father. 

Hysterical  and  Hysteroid  Conditions. — In  other  cases  one 
observes  hysterical  attacks  or  symptoms  of  nervous  weak- 
ness which  we  would  call  in  the  adult  cerebral  neurasthenia. 
Intercurrent  nightly  headaches,  sudden  attacks  of  dizziness, 
and  epileptic  seizures  direct  our  attention  to  the  true 
nature  of  this  nervous  condition. 

The  situation  becomes  especially  grave  if  a  change  in 
character  is  manifested,  when  along  with  mental  weakness 
there  are  sudden  outbursts  of  anger,  wilfulness,  and  rude- 
ness, or  a  retardation  in  the  mental  development  sets  in. 

These  psychic  anomalies  usually  appear  after  the  second 
dentition.  In  two  cases  of  developing  young  girls  I  saw  a 
moral  idiocy  show  itself.  Both  cases  were  from  good  fami- 
lies and  were  brought  up  in  a  good  social  environment,  yet 
they  consorted  with  men,  stole,  and  lied.  Confinement  in  a 
reform  school  did  not  improve  them.  Stigmata  of  congeni- 
tal lues  were  lacking,  the  physical  development  did  not  re- 
veal any  anomalies,  and  the  intelligence,  in  so  far  as  ad- 
vancement in  school  was  concerned,  was  normal.  In  one  of 
21 


322  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

the  cases  the  father  had  contracted  syphilis  eight  years 
before  the  birth  of  his  daughter,  in  the  other  ten  years' 
before. 

Migraine  May  Also  be  an  Expression  of  Congenital  Syphilis. 
— lialban  devotes  considerable  space  to  the  question  of 
symptomatic  hemicrania  in  his  monograph  on  juvenile 
tabes.  He  believes  that  without  paresis  and  tabes,  congeni- 
tal lues  can  cause  specific  migraine  attacks ;  in  other  words, 
hereditary  lues  during  puberty  may  find  its  only  manifesta- 
tion in  hemicrania.  I  have  observed  a  number  of  such  cases 
in  which  the  attacks  of  migraine  were  relieved  by  the  ad- 
ministration of  potassium  iodid. 

Psychoses. — The  psychoses  which  occur  in  acquired  lues 
are  found  also  in  congenital  lues. 

Symptoms  of  Hydrocephalus. — Symptoms  of  hydroceph- 
alus  appear  sometimes  in  the  form  of  disturbances  of  con- 
sciousness, mydriasis,  or  myosis  with  slowness  of  the  light 
reaction,  vomiting,  bradycardia,  partial  and  general  con- 
vulsions, pareses,  and  muscular  rigidity,  which  may  increase 
to  contractures. 

Symptoms  of  Arteritis.— Arteritis  causes,  as  in  adults, 
conditions  of  hemiparesis  and  paralysis,  which  may  be 
ushered  in  by  unilateral  or  general  convulsions  and  lead 
to  contractures  of  various  kinds  or  degrees.  There  may  or 
may  not  be  loss  of  consciousness.  The  same  is  true  in 
regard  to  aphasia. 

Mental  Weakness  and  Idiocy. — Diminution  of  the  intelli- 
gence in  slight  and  severe  degree,  comparatively  often 
reaching  idiocy,  is  a  frequent  occurrence  in  congenital  luetic 
disease  of  the  nervous  system. 

Bary  reports  six  cases  of  idiocy  or  dementia  due  to 
hereditary  syphilis.  Such  cases  must  in  my  opinion  be 
considered  as  cases  of  cerebral  lues,  in  which  idiocy  is  only 
a  symptom. 

This  was  true  in  the  case  of  Spielmeyer's.  Three  chil- 
dren of  a  father  who  had  been  syphilitic  were  taken  sick 
with  epileptic  convulsions,  rapidly  developing  dementia 
and  blindness.  The  .autopsy  in  one  of  the  children  who 
died  revealed  diffuse  disease  of  the  central  nervous  system. 


HEREDITARY  SYPHILIS  323 

Binswanger  found  in  74  cases  of  idiocy  that  lues  could 
be  demonstrated  in  the  father  in  seven  with  absolute  cer- 
tainty, and  with  probability  in  nine. 

Ziehen  found  congenital  lues  in  10  per  cent,  of  his  cases 
of  idiocy  as  certain,  and  as  probable  in  17  per  cent.  He 
reports  the  case  of  a  feeble-minded  girl  thirteen  years  old, 
with  loss  of  the  knee-jerks.  Under  energetic  antispecific 
treatment  the  knee-reflexes  returned,  as  well  as  the  intelli- 
gence. 

Epilepsy. — Epilepsy  in  its  various  forms  is  quite  frequent 
in  congenital  syphilis. 

Comprehensive  statistics  concerning  the  frequency  of 
the  relationship  between  hereditary  lues  and  epilepsy  are 
lacking.  Veit,  in  the  epileptic  institution  at  Wiihlgarten, 
found  this  relationship  existing  there  in  7  per  cent,  of  the 
cases.  Bratz  and  Luth,  in  400  epileptic  children  found  in 
5  per  cent,  of  the  cases  syphilis  in  the  parents.  In  these 
statistics  the  cases  with  multiple  brain  symptoms  and  con- 
vulsions are  excluded. 

The  epilepsy  either  appears  as  symptomatic,  as  the 
expression  of  organic  disease  of  the  brain,  or  as  the  so-called 
idiopathic  epilepsy. 

In  all  cases  of  epilepsy  either  with  or  without  mental 
feebleness  in  children  one  should  always  consider  the  possi- 
bility of  a  hereditary  luetic  basis.  Binswanger,  in  his 
monograph  concerning  epilepsy,  especially  emphasizes  the 
point  that  congenital  lues  is  a  much  more  frequent  etiologi- 
cal  factor  in  the  causation  of  epilepsy  than  has  generally 
been  supposed.  He  speaks  of  a  dyscrasic  form  of  congenital 
syphilitic  epilepsy,  in  rachitic,  scrofulous,  anaemic  children 
in  whom  lues  in  the  anamnesis  is  frequently  revealed  as  the 
cause.  In  such  cases  there  exists  a  germ  damage  or  injury 
in  contradistinction  to  a  germ  infection,  which  is  distin- 
guished by  the  pathology. 

One  of  my  cases,  a  child  eight  years  old,  the  father, 
several  years  before  its  birth,  had  been  syphilitic.  This 
child  was  normal  in  both  mental  and  physical  development, 
with  the  exception  of  some  backwardness  in  learning  to  talk. 
In  its  seventh  year,  without  any  apparent  cause,  epileptoid 


324  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

attacks  set  in,  which  gradually  developed  into  true  epileptic 
attacks.  Symptoms  of  congenital  lues  were  lacking  on  the 
skin,  mucous  membrane,  and  bones,  but  as  stigmata  of  lues 
of  the  nervous  system  there  were  found  double  mydriasis 
and  loss  of  the  pupil  light  reactions. 

Under  mixed  treatment  (mercury  and  potassium  iodid) 
the  epileptic  attacks  became  less  frequent.  At  the  end  of 
a  year  the  child  passed  from  under  my  observation. 

Basilar  Cranial  Nerve  Paralyses. — Paralyses  of  individ- 
ual cranial  nerves  may  be  the  only  symptoms  presented.  In 
a  case  reported  by  Grafes,  of  a  two-year-old  child  who  be- 
came blind  through  a  specific  iritis,  there  developed,  several 
weeks  before  it  came  under  Grafes's  observation,  a  left- 
sided  ptosis.  Objectively  there  was  found  a  divergent 
strabismus  and  mydriasis  on  the  left  side,  together  with  a 
papular  syphilide.  Antispecific  therapy  influenced  only  the 
syphilide.  The  postmortem  revealed  an  area  of  softening 
in  the  left  corpus  striatum  and  in  the  right  hemisphere, 
as  well  as  gummatous  tumors  in  the  sheath  of  the  left 
oculomotor  nerve. 

Paralysis  of  the  Eye-muscles  Less  Frequent  in  the  Congenital 
Than  in  Acquired  Lues.^Cases  of  paralysis  of  the  eye- 
muscles  are  in  general  less  frequent  in  congenital  than  in 
acquired  lues. 

The  paralysis  of  both  the  internal  and  external  eye- 
muscles,  with  hemi-  and  paraplegias,  as  well  as  with  demen- 
tia or  idiocy,  appears  relatively  often. 

Isolated  Loss  of  the  Light  Reaction. — The  loss  of  the  light 
reaction  of  the  pupil  has  been  observed  also  in  congenital 
as  in  acquired  lues  as  the  only  somatic  symptom. 

Finklenberg  has  reported  the  case  of  a  boy  eight  years 
old  who  presented,  as  the  only  objective  symptoms  of  an 
inherited  syphilis,  mydriasis  and  loss  of  the  light  reaction. 
The  boy  up  to  that  time  had  been  healthy.  The  father  of 
the  boy  had  syphilis,  and  later  tabes.  The  mother  had 
aborted  several  times  and  also  given  birth  to  feeble  children. 

As  an  accompaniment  of  other  brain  symptoms  a  dis- 
turbance of  the  function  of  the  pupil  occurs  very  often. 
Konig  considers  the  loss  of  the  light  reaction,  together  with 


-    HEREDITARY  SYPHILIS  325 

mental  feebleness,  with  very  rare  exceptions  as  a  certain 
sign  of  hereditary  syphilis. 

Polyuria  and  Glycosuria. — Demme  reports  a  case  of 
polyuria  and  transient  glycosuria  in  a  boy  six  years  old, 
which  disappeared  and  then  reappeared  after  a  year.  Both 
of  these  conditions  were  entirely  relieved  by  antispecific 
treatment.  Demme  describes  the  case  as  one  of  hereditary 
syphilitic  basilar  meningitis. 

The  Optic  Nerve. — The  optic  nerve  is  also  frequently 
involved  in  congenital  luetic  basilar  meningitis.  Oppen- 
heim  has  directed  attention  to  the  fact  that  along  with 
specific  basilar  meningitis,  which  in  acquired  lues  is  the 
chief  cause  of  neuritis  and  atrophy  of  the  optic  nerve,  in 
congenital  lues  hydrocephalus  also  appears  as  a  not  infre- 
quent etiological  factor. 

The  following  case  is  one  of  hereditary  syphilitic  disease 
of  the  optic  nerve : 

The  patient  was  a  boy,  fifteen  years  old,  who  was  born 
as  the  only  living  child  after  a  number  of  miscarriages  and 
still-born  children.  The  father  was  treated  for  syphilis  re- 
peatedly, the  last  time  about  one  year  ago.  The  child  was 
from  the  beginning  a  weakling.  He  learned  to  walk  late 
and  was  late  in  teething.  He  began  to  talk  first  when  he 
was  three  years  old,  and  at  six  he  talked  with  difficulty. 
From  this  time  on  his  mental  development  remained  station- 
ary. Convulsions  or  epileptic  attacks  never  occurred. 
When  the  boy  was  fourteen  he  became  blind,  in  the  course 
of  several  months,  in  the  right  eye.  Two  months  ago  the 
sight  of  the  left  eye  began  to  fail.  Objectively  the  cranium 
was  remarkably  small  and  lacking  in  symmetry.  There 
were  also  Hutchinson  teeth,  anisocoria,  complete  paralysis 
of  the  pupils,  on  the  right  side,  a  total  neuritic  atrophy,  and 
on  the  left  side,  in  addition  to  an  acute  optic  neuritis,  there 
was  a  disseminated  chorioiditis,  as  well  as  dust-like 
opacities  in  the  lens. 

In  this  case,  because  of  the  appearance  of  the  choroid 
and  lens  without  the  anamnesis,  the  diagnosis  of  a  congeni- 
tal specific  affection  of  the  optic  nerve  could  have  been  de- 
termined. Antispecific  treatment  caused  the  optic  neuritis 


326  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

to  recover  and  limited  the  atrophy.  A  year  has  since 
elapsed  with  no  return  of  the  neuritis. 

Deafness. — The  only  symptom  of  congenital  lues  may 
show  itself  in  difficulty  of  hearing  and  deafness.  Apart 
from  the  fact  that  the  cause  of  deafness  can  be  specific  dis- 
ease of  the  labyrinth,  the  acoustic  nerve  may  also  be 
affected,  as  observations  of  Fournier,  Siemerling,  and 
Bottinger  show. 

Complicated  Cerebrospinal  Symptoms. — Complicated  clini- 
cal pictures  are  presented  by  extensive  and  multiple  proc- 
esses in  the  brain  and  spinal  cord.  The  multiple  patho- 
logical processes  of  meningitis,  gummata,  cerebral  and 
spinal  endarteritis,  with  their  consequent  areas  of  soften- 
ing, correspond  in  the  clinical  sphere  to  headache,  dizziness, 
and  vomiting,  epileptic  attacks,  and  hemipareses  and 
paralyses,  disturbances  of  co-ordination,  anomalies  in  the 
behavior  of  the  tendon-reflexes,  as  well  as  defects  of  intelli- 
gence. 

In  general,  one  can  say  that  the  classification  of  Oppen- 
heim  into  arteritis,  meningitis  of  the  base  and  convexity, 
and  syphilitic  cerebro spinal  meningitis,  may  be  applied  to 
congenital  as  well  as  acquired  lues.  It  is  to  be  remembered, 
however,  that  a  combination  of  the  various  forms  occurs 
more  frequently  in  the  congenital  type. 

Hereditary  Syphilitic  Pseudoparalyses. — It  is  well  known 
that  Wegner's  osteochondritis  produces  epiphysial  pa- 
ralyses. So  long  as  this  does  not  occur,  just  so  long  are 
there  no  symptoms.  First,  when  the  separation  of  the 
epiphysis  occurs  as  a  result  of  the  necrotic  destruction  of 
the  calcification  zone  and  the  caseation  of  the  granulation 
tissue  in  the  medullary  spaces,  then  symptoms  in  the  form 
of  swelling  and  pain  appear  in  the  epiphysial  regions,  as 
well  as  deformities  of  the  bones. 

This  irritation  of  the  periosteum  and  swelling  of  the 
epiphysial  regions  occurs  most  frequently  at  the  upper 
epiphysis  of  the  humerus. 

If  the  clinical  picture  of  Parrott's  pseudoparalysis  is 
well  developed,  the  affected  child  is  unable  to  move  the 
arms  or  the  legs.  All  the  extremities,  or  some,  or  only  one, 


HEREDITARY  SYPHILIS  327 

may  be  involved.  The  paralysis  appears  as  a  flaccid  one. 
The  raised  arm  or  leg  falls  as  if  it  were  paralyzed  from  an 
acute  anterior  poliomyelitis. 

In  contradistinction  to  this  latter  paralysis,  however, 
two  things  may  be  noted:  first,  the  change  in  the  electrical 
reactions  of  both  nerve  and  muscle  are  lacking  in  the 
paralyzed  parts;  second,  passive  motion  causes  pain  and 
betrays  a  painful  swelling  on  the  end  of  the  bones  and  a 
crepitation  in  the  region  of  the  epiphyses,  as  well  as  the 
true  nature  of  the  paralysis.  In  the  upper  extremities  a 
slight  flexion  and  extension  in  the  fingers,  in  the  lower 
extremities  in  the  hip-joint,  even  in  the  severe  cases,  is 
preserved. 

The  paralysis  in  such  cases  is  due  to  two  causes:  first, 
the  muscle-tendons  have  no  fixed  point  of  insertion  since 
the  epiphyses  are  separated  from  the  diaphyses  and  slip 
around  here  and  there,  and,  second,  the  movements  of  the 
child  because  of  the  inflamed  periosteum  are  painful.  For 
this  reason  the  name  "pseudoparalysis"  seems  to  be  a 
fitting  one. 

This  affection  is  a  disease  of  the  first  weeks  of  life. 
Heubner  reports  a  case  as  occurring  at  the  end  of  the  fifth 
month,  but  such  exceptions  are  rare. 

There  are,  however,  not  always  tenderness  and  pain 
when  the  extremities  are  moved,  and  sometimes  the  clinical 
picture  makes  the  impression  of  a  genuine  paralysis. 

Reuter  expresses  the  opinion  that  a  real  paralysis  does 
exist  in  these  cases,  due  to  nerve  compression  produced 
by  periosteal  specific  exostoses  and  swellings  on  the  bony 
extremities.  Pollack  also  considers  that  a  true  paralysis 
occurs,  but,  contrary  to  Reuter,  assumes  an  affection  of  the 
central  nervous  system. 

Scherer  regards  it  as  probable  that,  just  as  in  other 
chronic  infectious  diseases,  so  also  here,  as  a  result  of  the 
poisoning  of  the  system  by  a  toxin,  paresis  or  paralysis  can 
develop  in  congenitally  syphilitic  children,  in  whom  no  bony 
changes  can  be  demonstrated  and  the  central  and  peripheral 
nervous  system  still  appear  to  be  intact.  The  etiology  of 
the  paralysis  may  be  attributed  to  either  the  toxic  effect  of 


328  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

the  Spiroclicete  pallida  or  to  other  forms  of  bacterial  life 
circulating  in  the  blood. 

Differential  Diagnosis. — In  considering  the  differential 
diagnosis  of  the  nervous  affections  caused  by  congenital 
syphilis  one  must  keep  constantly  in  mind  that  alcohol  pro- 
duces a  number  of  nervous  conditions  similar  to  some  of 
those  which  are  found  in  hereditary  lues.  Epilepsy,  idiocy, 
and  retardation  in  development  of  various  kinds,  as  well 
as  general  nervousness,  hysteria,  neurasthenia,  and  the  sim- 
ple psychoses,  are  common  to  both. 

Alcoholism  of  the  Parents. — How  similar  in  many  ways 
the  effect  of  alcoholism  in  the  parents  to  that  of  syphilis  is 
on  the  offspring,  the  work  of  Dr.  Sullivan,  of  Liverpool, 
shows  very  clearly.  iSullivan  began  making  observations 
in  the  large  prisons  of  Liverpool  of  the  children  of  female 
alcoholics.  After  the  exclusion  of  all  cases,  so  far  as  pos- 
sible, in  which  other  factors,  such  as  syphilis,  tuberculosis, 
and  nervous  degeneration,  might  enter  in  and  confuse 
the  deduction,  and  after  an  examination  of  hundreds  of 
cases  of  pure  alcoholism,  he  came  to  the  following  con- 
clusions : 

1.  Maternal  alcoholism  is  particularly  disastrous  for  the 
life  and  development  of  the  offspring. 

2.  The  influence  of  maternal  alcoholism  manifests  itself 
in  the  great  mortality,  in  the  tendency  to  still-births  and 
premature  births,  and  in  the  frequency  of  epilepsy  in  the 
living  children. 

3.  The  influence  of  the  alcohol  is  partly  that  of  a  poison 
acting  upon  the  maternal  organism  and  partly  from  a  direct 
toxic  effect  upon  the  embryo. 

A  fundamental  difference  exists,  however,  between  the 
influence  of  alcohol  and  syphilis.  In  syphilis  of  the  parents 
usually  the  first-born  are  the  most  severely  affected,  and  in 
the  subsequent  births  there  is  apt  to  be  a  gradual  lessening 
of  the  toxic  effect,  while,  on  the  other  hand,  in  alcoholism 
the  reverse  is  as  a  rule  true.  The  first-born  are  normal. 
Then  come  abnormal  children  which  survive  the  period  of 
infancy,  then  children  dying  prematurely,  then  still-births, 
and  finally  abortions. 


HEREDITARY  SYPHILIS  329 

It  not  infrequently  happens  that  both  alcoholism  and 
syphilis  exist  in  the  parents,  and  in  such  cases  it  is  often 
difficult  to  say  which  factor  should  receive  the  most  blame. 

Tuberculosis  of  the  Central  Nervous  System. — The  differen- 
tial diagnosis  between  cerebrospinal  syphilis  and  cerebro- 
spinal  tuberculosis  comes  oftener  into  consideration  in  chil- 
dren in  those  cases  in  which  basilar  luetic  meningitic  symp- 
toms develop  acutely,  and  in  which  congenital  syphilitic 
stigmata  have  either  not  been  present  or  were  not  observed, 
because  cerebrospinal  tuberculosis  occurs  more  often  in 
children  than  in  adults. 

The  combination  of  tuberculosis  and  syphilis  in  the 
parents  is  a  comparatively  frequent  one,  and  the  congeni- 
tally  luetic  children,  because  of  lessened  resistance,  yield 
easier  to  tuberculosis. 

As  the  prognosis  of  hereditary  luetic  visceral  affections 
in  general  improves,  the  later  the  tertiary  processes  develop 
in  the  internal  organs;  so  also  is  this  true  for  congenital 
syphilitic  disease  of  the  nervous  system.  One  sees  not  in- 
frequently cases  in  which  timely  antispecific  treatment 
brings  the  disease  to  quiescence,  yet,  as  in  acquired  lues, 
the  possibilities  of  relapses  and  intercurrent  exacerbations 
should  not  be  lost  sight  of. 

Metasyphilitic  Diseases  of  the  Nervous  System  in  Congenital 
Lues.  Tabes  Dorsalis. — Cases  of  actual  tabes  occur  in  con- 
genital lues.  Dydynski,  in  a  critical  study  of  this  subject, 
states  that  many  of  the  cases  which  have  been  described  as 
tabes  dorsalis  should  have  been  classified  as  Priedreich's 
ataxia.  The  presence  of  ataxia  and  Westphal's  symptom 
does  not  justify  the  diagnosis  of  tabes. 

The  following  cases  because  of  congenital  lues  were 
diagnosed  as  tabes: 

A  girl,  thirteen  years  old,  was  referred  to  me  on  account 
of  beginning  double  optic  atrophy.  The  father  had  a 
chancre  before  his  marriage  and  received  antispecific  treat- 
ment. Previous  to  the  birth  of  this  child  the  mother  had 
aborted  a  number  of  times.  The  child  had,  in  addition  to 
the  optic  atrophy,  double  mydriasis  with  loss  of  the  pupil- 
reflexes,  slight  sensory  disturbances  for  pain  in  the  lower 


330  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

extremities,  the  patellar  reflexes  were  only  obtainable  by 
reinforcement,  and  there  was  a  slight  ataxia  in  the  lower 
extremities.  There  were  no  evidences  of  tertiary  lues. 

A  laborer,  thirty-two  years  old,  was  married  six  years 
ago.  He  stated  that  before  his  marriage  he  had  never  had 
intercourse.  His  wife  stated  also  that  before  her  marriage 
she  was  a  virgin.  In  the  six  years  of  their  marriage  three 
children  were  born,  all  of  whom  were  weak,  but  at  the  time 
of  my  examination  presented  no  traces  of  hereditary  lues. 
One  year  ago,  without  any  apparent  cause,  the  patient  was 
taken  sick  with  lightning  pains  in  the  lower  limbs,  weakness 
of  the  bladder,  para3sthesias  in  the  toes  and  feet,  and  un- 
steadiness in  standing  and  walking.  The  patient  was 
always  temperate,  denied  excesses  in  venery,  and  had  never 
been  subject  to  severe  exposure. 

The  objective  examination  revealed  anisocoria  of  the  pu- 
pils, loss  of  the  pupil  light  reaction  with  accommodation  re- 
tained, absence  of  the  patellar  reflexes,  Eomberg's  symp- 
tom, hypalgesia  and  slowness  in  pain  conduction.  On  the 
left  leg  there  was  found  an  extensive  scar.  The  patient 
stated  that  when  nine  years  old  he  had  been  operated  on, 
in  the  Altona  City  Hospital,  for  a  bone  disease.  An  exam- 
ination of  the  records  there  showed  that  his  bone  affection 
had  at  that  time  been  finally  diagnosed  as  syphilitic  and 
was  healed  with  mercury  and  iodid  administered  inter- 
nally. The  record  also  stated  that  the  patient's  father  had 
syphilis. 

At  the  present  time  many  cases  of  infantile  and  juvenile 
tabes  as  a  consequence  of  congenital  syphilis  have  been  re- 
ported, to  the  correct  diagnosis  of  which  no  objection  can  be 
made.  Halban  and  Dydynski  direct  attention  to  the  fre- 
quent and  early  appearance  of  optic  atrophy  and  partial 
bladder  paralysis,  as  well  as  to  the  rareness  of  severe  ataxia 
in  juvenile  tabes.  Otherwise,  all  the  symptoms  of  tabes 
of  the  adult,  including  the  crises  and  trophic  disturbances, 
occur  in  the  congenital  form. 

In  cases  of  infantile  and  juvenile  tabes  the  frequency  of 
occurrence  in  the  anamnesis  of  postsyphilitic  disease  in 
the  family  is  worthy  of  mention. 


HEREDITARY  SYPHILIS  331 

Pseudotabes  Syphilitica. — As  one  must  be  on  his  guard 
not  to  overlook  an  extra-uterine  acquired  lues,  so  one  must 
not  diagnose  a  non-tabetic  ataxia  in  childhood  as  a  tabes. 

It  is  to  be  remembered  that  individual  tabetic  symptoms 
may  be  exhibited  by  atypical  genuine  specific  disease  of  the 
posterior  columns. 

Dementia  Paralytica. — Dementia  paralytica  also  occurs  as 
a  result  of  congenital  lues,  and  recently  the  cases  appear- 
ing in  the  literature  have  been  more  frequent  since  our 
attention  has  been  more  sharply  directed  to  this  condition 
by  several  pertinent  observations. 

A  case  of  my  own  is  especially  interesting,  since  here  the 
dementia  paralytica  was  the  only  expression  of  hereditary 
syphilis,  in  a  boy  twelve  years  old,  and  also  because  the 
luetic  history  was  rather  unusual. 

The  father  absolutely  denied  ever  having  had  syphilis 
and  a  painstaking  examination  revealed  no  past  or  present 
evidences  of  it.  The  mother  stated  that  while  taking  care 
of  a  luetic  patient  she  contracted  the  disease  extragenitally. 
She  married  several  years  later,  after  having  taken  anti- 
specific  treatment. 

Her  first  pregnancy  terminated  with  the  birth  of  a  dead 
foetus  at  the  sixth  month.  The  second  child  was  born  a 
month  too  early,  developed  in  the  fourth  month  of  life  a 
rash,  and  died  in  the  beginning  of  the  second  year.  This 
boy  was  the  third  child,  who  likewise  was  born  a  month  too 
early.  A  fourth  child  was  born  at  full  term  and  since  his 
fourth  year  has  been  afflicted  with  paroxysmal  hsemoglo- 
binuria.  Finally  a  fifth  child  was  born  at  full  term  as  a 
normal  baby,  and  this  child  remained  healthy  until  sixteen 
months  old,  when  it  died  with  vomiting  and  diarrhoea. 

This  boy  remained  apparently  well  until  in  his  eleventh 
year,  when  a  progressive  and  rapid-developing  dementia  set 
in,  which  was  accompanied  by  an  articulatory  speech  dis- 
turbance, tremor  of  the  extremities,  paresis  of  the  mimetic 
innervation,  increase  of  the  tendon-reflexes,  mydriasis,  and 
loss  of  the  pupil-reflexes. 

In  the  cases  reported  in  the  literature  syphilis  of  the 
parents  has  not  always  been  demonstrated.  In  some  of  the 


332  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

cases  a  specific  infection  was  found  to  have  occurred  in 
infancy  and  childhood.  In  others  there  was  a  combination 
of  several  etiological  factors,  such  as  severe  psychopathic 
inheritance,  alcoholism  of  one  or  both  parents,  and  head 
injuries. 

It  can  be  said  at  the  present  time  that  both  tabes  and 
dementia  paralytica  as  a  consequence  of  hereditary  lues  are 
found  in  all  stations  of  life.  The  following  case  is  a  classi- 
cal one  of  juvenile  paresis: 

A  girl,  fourteen  years  old,  presented  a  typical  picture  of 
dementia  paralytica,  which  began  when  she  was  eight  years 
old.  The  father,  during  his  courting  period,  infected  his 
bride  through  kissing  with  a  lip  chancre.  He  later  died  with 
general  paresis.  The  mother  presents  at  the  present  time 
the  clinical  picture  of  an  abortive  tabes.  The  girl  died,  and 
the  autopsy,  as  well  as  the  microscopical  examination, 
showed  the  pathology  of  paresis  along  with  a  typical 
Heubner  's  endarteritis. 

The  course  of  infantile  paresis  is,  on  the  average,  a 
longer  one  than  it  is  in  adults.  Alzheimer  places  the  aver- 
age duration  as  four  and  a  half  years.  In  the  majority  of 
the  cases  inferior  mental  endowment  dated  back  to  infancy; 
also  a  simple  progressive  dementia  was  the  type  most  often 
presented  and  the  depressive  and  maniacal  states  so  often 
seen  in  a  classical  paresis  were  lacking.  Severe  paralysis 
is  particularly  inclined  to  develop  early  and  paralytic 
attacks  are  more  liable  to  occur  in  the  beginning  of  the 
disease.  Tabetic  symptoms  are  also  more  frequently  found. 

The  pathology  does  not  differ  from  the  pathology  in  the 
adult  form.  The  combination  of  the  typical  findings  in 
paresis  along  with  Heubner 's  endarteritis  is  a  frequent  one. 

In  addition  to  tabes  and  paresis  other  clinical  pictures 
are  presented  in  children  which  must  be  regarded  as  meta- 
or  postsyphilitic  affections. 

Homen's  Familiar  Disease  (Idiocy  with  Spastic  Paralysis). — 
Homen,  in  1892,  described  a  disease  appearing  in  three  chil- 
dren of  the  same  family,  which  was  evidently  due  to  con- 
genital lues.  This  affection  begins  usually  between  the 
ages  of  twelve  and  twenty,  with  symptoms  of  dizziness, 


HEREDITARY  SYPHILIS  333 

heaviness  in  the  head,  and  disturbances  of  the  general 
health;  then  the  intelligence  and  memory  begin  to  fail. 
Along  with  vague  and  diffuse  pains  in  the  limbs,  the  gait 
becomes  uncertain  and  the  speech  difficult.  The  loss  of  the 
intelligence  progresses  to  idiocy.  The  speech  disturbance 
resembles  in  nature  to  the  last  more  that  of  a  lack  of  initia- 
tive than  anything  else.  Spastic  symptoms  appear  in  the 
lower  limbs  and  then  in  the  upper,  progressing  to  well- 
developed  contractures.  Somatic  symptoms  on  the  part  of 
the  pupils  or  in  the  form  of  anaesthesias  or  pareses  were 
absent.  There  existed  in  all  three  children  a  certain  degree 
of  infantilismus. 

The  pathology  of  all  three  cases  was  similar. 

Macro  scopically  there  were  a  thickening  of  the  cranium, 
the  dura  and  pia  mater,  atrophy  of  the  brain  in  its  entirety, 
sclerosis  of  the  cerebral  vessels,  areas  of  softening,  and  cir- 
rhosis of  the  liver.  Microscopically  there  were  absorption 
of  the  tangential  fibres,  atrophic  changes  in  the  large  py- 
ramidal cells,  thickening  of  the  neurologia,  and  arterioscle- 
rotic  and  hyaline  changes  in  all  the  cerebral  vessels.  Homen 
regarded  the  changes  in  the  vessels  as  the  most  essential 
and  primary  part  of  the  process. 

Spastic  Spinal  Paresis  with  and  without  Cerebral  Symptoms. 
— In  1892  Friedmann  published  two  cases  of  relapsing, 
apparently  luetic  so-called  spinal  paralysis  occurring  in 
childhood.  These  cases  occurred  in  two  boys,  ten  and  fifteen 
years  old,  respectively.  In  the  first  case,  after  two  attacks, 
under  antispecific  therapy  complete  recovery  took  place 
both  times.  In  the  second  case,  also  recovery  after  the  first 
attack  occurred,  but  after  the  second  the  gait  was  only 
improved. 

Upon  the  basis  of  these  observations,  which  were  simply 
clinical  ones,  Friedmann  concluded  that  there  is  a  typical 
form  of  spastic  paraplegia  in  children  which  is  to  be  dis- 
tinguished from  the  so-called  congenital  spastic  paraplegia ; 
also  that  this  form  is  due  to  congenital  lues  and  clinically 
is  defined  by  its  tendency  to  complete  recovery,  relapses, 
and  the  absence  of  brain- symptoms.  Friedmann  regards 
this  type  of  spinal  paralysis  in  children  as  similar  to  the 


334  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

syphilitic  spinal  paralysis  of  Erb  in  adults.  Hoffman,  in 
1894,  also  reported  a  similar  case  to  that  of  Homen's. 

The  following  case  came  under  my  observation : 

A  seven-year-old  girl  was  born  at  the  seventh  month  and 
with  great  difficulty  made  to  live.  She  remained  feeble, 
learned  to  talk  late,  teethed  late  and  badly,  and  became  clean 
in  her  habits  later  in  life  than  usual.  She  never  learned  to 
walk,  but  had  a  spastic  paresis  in  the  lower  extremities. 
Treatment  with  plaster-of-Paris  bandages  caused  no 
improvement. 

Her  father,  three  years  before  his  marriage,  had  a  hard 
chancre,  which  was  healed  by  administration  of  mercury 
and  iodid. 

The  girl  had  the  appearance  of  an  imbecile.  There  was 
a  left  convergent  strabismus,  but  otherwise  no  oculopupil- 
lary  anomalies.  The  cranium  was  slightly  microcephalic, 
the  teeth  much  decayed  and  abnormal.  The  lower  extremi- 
ties were  spastic  and  paretic.  Disturbances  of  sensation 
and  of  the  sphincters  were  absent.  The  child  was  unable  to 
stand  or  walk.  The  speech  was  normal.  Syphilitic  stig- 
mata were  lacking. 

Sachs's  Amaurotic  Idiocy. — The  interesting  work  of  Sachs 
should  be  mentioned  here.  He  has  described  a  clinical  syn- 
drome which  likewise  consisted  in  dementia  and  spastic 
paralysis  of  the  extremities.  In  addition  to  these  symptoms 
there  was  also  a  failure  in  power  of  vision,  which  gradually 
progressed  to  complete  blindness.  This  affection  appeared 
in  several  members  of  the  same  family.  The  pathology  was 
that  of  a  disease  of  the  cells  and  medullary  fibres  of  the 
brain  cortex,  corresponding  to  that  of  retarded  develop- 
ment. Sachs  did  not  attribute  any  relationship  between 
these  cases  and  syphilis.  There  was  no  suspicion  of  syph- 
ilis in  the  history  and  there  were  no  changes  in  the  blood- 
vessels or  inflammatory  symptoms  of  that  nature  to  be 
found.  He  assigns  this  affection  to  that  category  in  which 
an  inherited  tendency  or  family  tendency  is  the  last  cause 
of  the  anomalies  in  the  nervous  system,  which  depend  either 
upon  intermarriages  or .  a  trauma  to  the  mother  during 
pregnancy. 


HEREDITARY  SYPHILIS  335 

Since  Sachs's  studies  concerning  this  affection  many 
cases  of  a  similar  nature  have  been  reported  in  the  litera- 
ture without  adding  anything  clinically  new  to  the  subject. 

Disseminated  Sclerosis. — Jacobson  has  discovered  a  case 
of  multiple  sclerosis  in  hereditary  lues  in  a  child  which 
developed  intra-uterine  an  interstitial  keratitis  and  at  the 
autopsy  there  were  found  hepatitis,  perihepatitis,  hyper- 
plasia  of  the  spleen,  perisplenitis,  and  parenchymatous 
nephritis.  There  were  also  found  in  the  brain,  both  in 
the  gray  and  white  matter,  numerous  gray  indurated  spots 
which  varied  in  size  from  a  mustard-seed  to  a  hazel-nut,  and 
which  were  evidently  of  a  different  character  from  the 
sclerotic  plaques  found  in  the  idiopathic  multiple  sclerosis. 
There  was  besides  an  extensive  leptomeningitis. 

The  clinical  course  was  a  different  one  from  that  of  the 
ordinary  multiple  sclerosis.  Beginning  with  headache  and 
fever,  a  left  hemiplegia  and  anisocoria  developed,  which, 
under  the  administration  of  potassium  iodid,  disappeared. 
There  was  present  also  an  athetotic-like  movement  in  the 
hands  and  fingers  on  both  sides.  There  then  appeared  sud- 
denly some  general  cerebral  symptoms,  from  which  the  boy 
died. 

In  a  case  published  by  Buchholz,  along  with  severe 
psychic  disturbances  there  were  intentional  tremor,  in- 
crease of  the  tendon-reflexes,  motor  weakness  and  spas- 
ticity  of  the  lower  extremities,  speech  disturbances  not  of 
the  scanning  kind,  anomalies  of  the  pupils,  optic  atrophy, 
but  no  nystagmus. 

The  autopsy  revealed  multiple  gummata,  syphilitic  dis- 
ease of  the  blood-vessels,  cavity-like  areas  in  the  substance 
of  the  brain,  thickening  of  the  cerebral  and  spinal  lepto- 
meninges,  as  well  as  a  diffuse  increase  of  the  supporting 
substance  in  the  spinal  cord.  Clinically  and  pathologically 
the  case  was  a  very  different  one  from  multiple  sclerosis. 

Symptoms  of  Friedreich's  Ataxia. — In  rare  cases  there  has 
been  observed  a  symptom-complex  in  congenital  lues  which 
resembles  the  one  presented  in  Friedreich's  ataxia.  Oppen- 
heim  reports  cases  in  which  he  was  unable  to  make  a  differ- 
entiation with  certainty.  Yet,  in  the  majority  of  these  cases, 


336  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

the  appearance  of  spastic  symptoms,  convulsions,  and 
paralysis  of  the  eye-muscles  serves  to  differentiate  the  true 
condition. 

Bayet  reports  the  history  of  a  family  in  which  four 
children,  in  age  from  nine  to  seventeen,  presented  the 
picture  of  a  Friedreich's  ataxia.  The  mother  had  aborted 
numerous  times  and  positive  symptoms  of  syphilis  were 
found  in  the  children.  Bayet  assumes  that  the  syphilis  had 
caused  a  cessation  in  the  development  of  the  cord. 

Congenital  Syphilis  in  the  Third  Generation. — In  conclusion, 
some  consideration  should  be  given  to  the  influence  of 
syphilis  on  the  third  generation,  whether  or  not  in  the 
offspring  of  congenitally  syphilitic  parents,  stigmata  of 
congenital  lues  are  demonstrable,  especially  in  the  nervous 
system. 

Numerous  observers  have  contributed  pertinent  experi- 
ences on  this  subject. 

Mensinger  reports  the  following  case : 

The  grandfather  had  contracted  lues  before  his  mar- 
riage. In  all  of  his  six  children,  either  at  or  soon  after 
their  birth,  symptoms  of  congenital  lues  were  manifested. 
One  of  these  children,  the  next  to  the  oldest  daughter,  at  the 
age  of  twenty  married  a  man  who,  after  an  examination 
by  a  physician,  was  pronounced  free  from  syphilis.  Of  the 
five  children  born  from  this  marriage  the  first  was  luetic, 
the  second  healthy,  the  third  became  sick  with  meningitis 
when  one  year  old,  which  was  cured  by  the  giving  of  potas- 
sium iodid,  the  fourth  and  fifth  children  were  also  syph- 
ilitic and  infected  their  wet-nurse. 

Barthelemy  cites  three  interesting  cases.  One  was  an 
idiot  born  of  a  congenitally  luetic  mother,  a  second  was  an 
epileptic  whose  father  died  with  general  paresis  and  whose 
grandfather  on  the  father's  side  had  syphilis.  The  third 
was  a  woman  who,  at  the  age  of  forty-three,  developed  a 
spastic  spinal  paralysis.  Both  of  the  parents  of  this 
woman  were  syphilitic.  The  woman  herself  had  a  mentally 
backward  daughter,  with  hydrocephalus,  asymmetrical 
head,  and  strabismus. 


HEREDITARY  SYPHILIS  337 

The  largest  material,  however,  comes  from  A.  Founder, 
who  has  collected  from  the  literature  45  cases,  in  18  of 
which  the  proof  of  inherited  syphilis  was  certain.  Fournier 
further  presents  46  marriages  of  congenital  luetics  from  his 
own  experience.  In  these  46  marriages  there  occurred  143 
pregnancies  and  these  ended  in  43  cases  in  abortions,  39 
cases  with  either  still-born  children  or  children  dying  soon 
after  birth,  and  in  63  cases  with  living  children.  In  this 
entire  material  there  were  found  dystrophies,  eye  defects, 
tumors  of  the  brain,  rachitis,  general  retardation  of  physical 
development,  infantilismus,  mental  and  idiotic  dystrophies, 
epilepsy,  and  hysteria,  nervous  convulsions,  neoplasms  of 
the  heart,  closure  of  the  urethra,  anomalies  of  the  fingers 
and  toes,  atrophy  of  the  tongue,  congenital  extremity  ampu- 
tations, and  neoplasms  on  the  palate,  ears,  and  lips. 

All  of  these  observations  show  how  far-reaching  the 
influence  of  inherited  lues  is  and  also  serve  to  emphasize 
the  importance  of  anamnestic  researches  and  inquiries 
where  inhibited  and  deficient  development  occurs  in  the 
nervous  system. 


22 


XVII 

CONCERNING  THE  BEHAVIOR  OF  THE  WASSER- 
MANN  REACTION  IN  THE  BLOOD  AND  SPINAL 
FLUID,  ALSO  PLEOCYT08IS  AND  THE  IN- 
CREASE OF  GLOBULIN  (PHASE  I)  IN  SYPHILO- 
GENETIC  DISEASES  OF  THE  NERVOUS  SYSTEM. 

Introduction. — The  results  of  investigations  as  to  (a) 
lymphocytes,  (b)  increase  of  albumin  in  the  cerebrospinal 
fluid,  and  (c)  the  existence  of  the  "VVassermann  reaction 
(W.  R.)  in  the  blood,  have  in  recent  years  been  used  by 
neurologists  of  all  countries  for  the  differential  diagnosis 
between  syphilitic  affections  and  other  diseases  of  the  cen- 
tral nervous  system.  These  attempts  have  greatly  advanced 
our  knowledge.  The  statements  of  the  French  (particularly 
of  Vidal,  Ravant,  and  iSicard)  have  been  generally  con- 
firmed. The  investigation  as  to  the  increase  of  albumin  in 
the  cerebrospinal  fluid,  introduced  by  Nissl,  has  been  made 
easy  by  the  so-called  " Phase  I"  reaction,  introduced  by 
myself  and  Apelt,  and  tested  by  us  on  a  large  number  of 
patients.  I  first  published  this  reaction  four  years  ago, 
and  its  value  has  since  been  confirmed  by  many  authors, 
so  that  it  may  now  be  regarded  as  generally  accepted  in  the 
diagnostic  methods  of  neurology. 

The  Wassermann  Reaction  Not  a  Specific. — The  W.  R.  in 
the  blood  can  no  longer  be  regarded  as  specific,  but  only 
as  characteristic  of  syphilis,  for  it  has  been  stated  by  many 
authors  that  it  also  occurs  in  a  certain  percentage  of  some 
tropical  diseases,  such  as  leprosy,  malaria,  and  in  recent 
cases  of  scarlatina. 

Examination  of  Cerebrospinal  Fluid. — The  examination  of 
the  cerebrospinal  fluid  for  the  W.  R.  has  become  of  greater 
importance  since  Hauptmann  and  Hoessli  in  my  clinic  in- 
troduced the  use  of  increasing  quantities  of  the  cerebro- 
spinal fluid.  I  have  also  tested  and  determined  the  value  of 

338 


BEHAVIOR  OF  THE  WASSERMANN  REACTION     339 

this  method  on  the  material  in  my  ward.  It  has  been  shown 
that  the  Wassermann  reaction  in  the  spinal  fluid,  in  larger 
quantities  of  the  fluid  up  to  1  c.c.,  occurs  in  syphilogenetic 
diseases  of  the  nervous  system.  On  the  other  hand,  this 
question  has  reached  a  certain  finality  in  so  far  that  any 
one  who  can  use  the  "four  reactions"  and  interpret  them 
correctly  may,  by  their  aid,  clearly  diagnose  cases  which 
are  clinically  doubtful. '  During  the  last  four  years  I  have 
occupied  myself  almost  uninterruptedly  with  these  reac- 
tions, as  they  from  time  to  time  appeared  in  the  literature, 
and  have  tried  to  solve  the  questions,  as  they  presented 
themselves,  from  the  abundant  material  at  my  disposal  in 
hospital  and  private  practice.  I  especially  tried  to  ascer- 
tain how  far  the  result  of  the  "four  reactions"  would  admit 
of  definite  conclusions. 

Of  the  numerous  comprehensive  studies  which  deal  with 
the  examination  of  the  spinal  fluid  as  a  diagnostic  agent, 
and  which  confirm  my  conclusions,  I  will  mention  only  those 
of  Rehm,  W.  Holzmann,  Eichelberg,  and  Stertz. 

The  Technique  of  Lumbar  Puncture. — The  technique  of 
lumbar  puncture  is  comparatively  simple,  and  is  as  follows : 
In  the  middle  of  a  line  connecting  the  crests  of  iliac  bones  a 
puncture  is  made  between  the  laminae  of  the  third  and 
fourth  lumbar  vertebrae  by  means  of  a  cannula  and  trocar. 
It  is  best  to  let  the  patient  lie  on  his  side  during  the  opera- 
tion. I  personally  prefer  to  let  him  lie  on  his  right  side. 
The  pressure  of  the  fluid  as  it  escapes  is  measured  by  a 
vertical  glass  tube,  graduated  in  mm.  The  pressure  is 
in  normal  adults  about  130  mm.  A  quantity  of  5  c.c.  of 
fluid  is  sufficient  for  the  purpose  of  testing  the  reactions, 
for  one  should  take  as  little  as  possible  to  avoid  disagree- 
able complications,  which,  however,  will  occur  now  and 
then  in  spite  of  all  precautions.  These  occasional  disagree- 
able consequences  consist  of  headache,  nausea,  and  vomit- 
ing. The  quieter  the  patient  is  kept  after  the  lumbar  punc- 
ture, the  less  will  be  the  liability  of  trouble  arising  from  this 
trifling  operation. 

Dangers  and  Unpleasant  After-effects  of  Lumbar  Puncture. — 
Experience  goes  to  show  that  paralytics  and  tabetics  are  in 


340  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

almost  all  cases  free  from  subsequent  troubles.  On  the 
other  hand,  nervous  and  hysterical  patients,  as  well  as 
healthy  persons,  often  experience  for  one  or  more  days 
disagreeable  symptoms.  The  withdrawal  of  spinal  fluid  in 
cases  of  cerebral  tumor  is  by  no  means  free  from  danger. 
Thirty  fatal  cases  have  been  reported  in  the  literature. 
Personally  I  have  seen  four  cases  of  sudden  death  in 
patients  with  cerebral  tumor  following  the  lumbar  punc- 
ture. In  about  3000  cases  of  lumbar  puncture  in  patients 
with  other  diseases  I  have  never  observed  any  permanent 
injurious  consequences.  As  a  precaution  it  is  advisable,  for 
twenty-four  hours  after  the  operation,  to  allow  the  patient 
to  lie  quietly  in  bed,  on  his  back,  with  head  low,  and  not 
allow  him  to  get  up  during  this  time,  even  for  the  perform- 
ance of  his  natural  functions.  If  one  omits  these  precau- 
tions, most  disagreeable  consequences  may  arise,  for  the 
patient  may  suffer  for  days,  and  even  weeks,  from  headache, 
giddiness,  and  nausea.  To  perform  the  puncture  in  the  con- 
sultation room  (as  has  been  done)  is  absolutely  to  be  con- 
demned, because  the  doctor  may  be  held  liable  for  any 
possible  disagreeable  results.  I  have  myself  experienced 
this  in  a  case,  to  my  sorrow. 

The  Presence  of  Blood  in  the  Cerebrospinal  Fluid. — The 
cerebrospinal  fluid  usually  flows  out  from  the  needle  or  can- 
nula  as  clear  as  water,  but  it  sometimes  happens,  in  spite 
of  the  most  skilful  technique,  that  a  small  vein  is  punctured 
and  some  blood  mixes  with  the  fluid.  The  presence  of  blood 
may  also  be  observed  in  the  spinal  fluid  in  subdural  hemor- 
rhage, fracture  of  the  skull,  cerebral  apoplexy,  etc.  It 
is  comparatively  easy  to  differentiate  between  a  recent  and 
an  old  hemorrhage.  In  recent  hemorrhage,  after  centrifug- 
ing,  the  spinal  fluid  becomes  as  clear  as  water.  Where  the 
hemorrhage  has  occurred  some  time  before,  the  spinal  fluid 
presents  a  light-yellow  appearance,  indicating  the  presence 
of  haemoglobin. 

A  mistake  as  to  whether  the  blood  is  caused  by  the 
lumbar  puncture,  or  comes  from  a  subdural  hemorrhage,  is 
scarcely  likely  to  occur,  since  one  can  distinguish  between 


the  blood  produced  by  the  puncture  and  other  recent  hemor- 
rhages from  the  clinical  symptoms  present  previous  to  the 
puncture. 

Both  Klieneberger  and  I  have  been  able  to  demonstrate 
a  yellowish  tinge  (xantochromia),  as  well  as  either  the 
presence  or  absence  of  a  moderate  degree  of  pleocytosis  in 
compression  of  the  spinal  cord.  This  behavior  of  the  spinal 
fluid  also  occurs  in  intramedullary  tumors  and  intramedul- 
lary  syphilis,  as  Eaven  and  I  have  shown  from  material  in 
my  department. 

It  goes  without  saying  that  such  fluid  is  useless  for  an 
estimation  of  the  increase  of  either  albumin  or  white  cells, 
but  a  slight  admixture  of  blood  does  not  interfere  with  the 
W.  R. 

The  Globulin  Test.  Phase  I. — The  simplest  method  of 
testing  the  cerebrospinal  fluid  for  albuminous  bodies  is 
to  search  for  an  increase  of  globulin — the  so-called  ' '  Phase 
I"  reaction,  introduced  by  myself  and  Apelt.  For  this 
purpose  there  is  added  to  a  hot  saturated  solution  of 
sulphate  of  ammonium  which  has  been  permitted  to  cool  an 
equal  quantity  of  fluid ;  1  c.c.  of  each  is  quite  sufficient.  It 
is  advisable  to  pour  one  liquid  gently  on  top  of  the  other; 
if  the  globulins  are  increased,  there  occurs  a  more  or  less 
distinct  gray  ring  at  the  plane  of  contact.  After  this  pre- 
liminary observation  the  mixture  is  well  shaken  and  the 
result  may  be  read  off  within  three  minutes.  If  it  is  dis- 
tinctly opalescent  or  cloudy,  we  call  it  a  positive  "Phase 
I"  reaction. 

The  Fuchs-Rosenthal  Method  of  Counting  the  Lymphocytes. 
— For  the  examination  of  the  cerebrospinal  fluid  as  to  an 
increase  of  cells,  we  have  found  the  Fuchs-Rosenthal  count- 
ing-chamber best.  It  is  somewhat  larger  than  the  one  gener- 
ally used  to  count  the  red  and  white  blood  corpuscles.  The 
counting  is  performed  as  follows :  The  staining  fluid  (0.1  G. 
methyl  violet,  2.0  G.  glacial  acetic,  50  G.  distilled  water) 
is  drawn  up  by  the  pipette,  in  general  use  for  counting  the 
white  blood-cells,  to  point  I,  and  then  further  filled  with 
the  liquor  to  point  XL  After  shaking  the  pipette  for  five 
minutes,  a  drop  of  the  mixture  is  put  on  the  counting- 
chamber.  One  counts  all  the  lymphocytes  and  leucocytes  in 


342  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

all  the  squares,  and  divides  the  number  ascertained  by 
three.  Our  experience  has  taught  us  that  one  may  regard 
from  0  to  5  cells  per  c.mm.  as  normal,  6  to  10  as  borderland, 
while  more  than  10  cells  per  c.mm.  constitute  pathological 
increase. 

Technique  of  Obtaining  Blood  for  the  Wassermann  Reaction. 
— The  technique  of  obtaining  the  blood  for  the  Wassermann 
reaction  is  very  simple.  A  "Bier"  ligature  is  tied  around 
the  upper  arm  so  firmly  that  the  veins  of  the  forearm  fill 
tensely,  but  the  pulse  at  the  wrist  is  still  palpable.  The 
cannula  or  needle  is  then  introduced  from  above,  so  that  the 
venous  blood  may  escape  in  its  natural  direction,  and  5  to 
10  c.c.  of  blood  are  taken.  The  blood  is  then  thoroughly 
stirred  with  a  glass  rod  to  facilitate  the  separation  of  the 
serum,  and  this  stirring  is  repeated  at  intervals  of  half  an 
hour  until  the  process  is  complete.  It  is  then  preserved 
on  ice  until  it  is  to  be  used  for  the  W.  R. 

The  Wassermann  Reaction. — I  do  not  intend  to  describe 
the  technique  of  the  W.  B.  in  detail.  It  must  be  performed 
in  large  institutions  or  in  private  special  institutions  on 
account  of  the  serological  training  and  the  number  of  con- 
trols required,  which  are  possible  only  in  such  places.  But 
I  consider  it  necessary  to  shortly  recall  the  history  and 
character  of  the  reaction.  Wassermann  started  from  the 
Bordet-Gengou  doctrine  of  the  complement-deviation  by 
known  micro-organisms,  and  tried  to  apply  this  phenom- 
enon to  diseases  the  organism  of  which  was  not  as  yet 
known,  or  at  any  rate  had  not  been  grown  artificially.  He 
used,  in  the  place  of  pure  cultures  of  micro-organisms, 
organs  which  either  may  or  may  not  with  certainty  contain 
the  organism  of  the  disease  in  question;  for  instance,  in 
syphilis,  the  liver  of  a  luetic  fretus  plentifully  pervaded  with 
spirochaetes.  After  having  completed  his  preliminary  in- 
vestigations, he  published,  together  with  Neisser  and  Bruck, 
the  well-known  reaction  by  means  of  which  one  can  prove 
that  an  individual,  at  some  time  during  his  life,  has  been 
infected  with  syphilis.  The  original  method,  after  the  test 
of  time,  has  proved  best,  although  several  authors  have 
proposed  modifications. 


BEHAVIOR  OF  THE  WASSERMANN  REACTION     343 

From  the  liver  of  a  luetic  foetus  a  watery  extract  is  pre- 
pared, and  if  this  is  added  to  the  serum  of  a  syphilitic 
individual  it  is  able  to  deflect  the  complement  from  the 
haemolytic  serum  (consisting  of  sheep's  blood-corpuscles 
and  guinea-pig  serum  as  "complement,"  and  of  the  serum 
of  a  rabbit  previously  treated  with  sheep's  blood  as  "ambo- 
ceptor"),  and  thus  to  impede  the  combination  of  the  sys- 
tem and  the  resultant  haemolysis. 

A  glance  at  the  following  will  enable  one  to  better  under- 
stand the  reaction. 

Wassermann-Neisser-Bruck  Syphilis-Reaction-Compo- 
nents.— (1)  Sheep's  blood-corpuscles,  (2)  amboceptor,  (3) 
complement-haemolytic  system,  (4)  antigen  (extract  from 
organ),  (5)  liquid  to  be  examined  (serum  or  cerebrospinal 
fluid)  containing  antibodies  (?). 

These  components  are  prepared  as  follows:  (1)  Sheep's 
blood-corpuscles  are  freed  from  all  serum  elements  by  cen- 
trifugation  with  sodium  chloride,  (2)  rabbit  serum  pre- 
viously treated  with  sheep's  blood,  (3)  freshly  obtained  nor- 
mal guinea-pig  serum,  (4)  watery  or  alcoholic  extract  of  a 
hereditary-luetic  liver  (or  of  a  normal  heart),  (5)  serum 
freshly  taken  or  kept  sterile  by  preservation  on  ice,  and 
obtained  by  simple  separation  from  the  blood  to  be 
examined. 

Principle  of  the  Reaction. — Two  interactions  ("ring") 
may  occur  between  these  components : 

(a)  Sheep's  blood-corpuscles  are,  in  the  presence  of 
complement,  dissolved  by  the  amboceptor  (hsemolytic 
system),  or  (b)  a  second  interaction  occurs  between  antigen, 
antibody,  and  complement. 

If  antibodies  exist  in  the  serum  to  be  examined  they 
attract  to  themselves,  in  conjunction  with  the  antigen,  the 
complement,  so  that  the  first  interaction  cannot  take  place 
from  want  of  complement,  and  consequently  the  blood- 
corpuscles  cannot  be  dissolved. 

1  and  2  and  3 — the  first  interaction. 

3  and  4  and  5  ( ?) — the  second  interaction. 

POSITIVE  REACTION. — 1  and  2  (3  participates  in  the  sec- 
ond interaction;  the  first  interaction  does  not  occur;  the 


344  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

blood-corpuscles  remain  undis solved) ;  3  and  4  and  5 — the 
second  interaction. 

NEGATIVE  REACTION. — 1  and  2  and  3 — the  first  interaction 
(haemolysis).  3  participates  in  the  first  interaction,  from 
the  second  interaction  only  4  is  present,  while  5  (antibodies) 
are  not  present  in  the  non-luetic  serum. 

Technique  of  the  Reaction. — The  serum  to  be  examined 
is  placed  for  half  an  hour  in  the  incubator  at  55°  C. 
(131°  F.)  to  destroy  its  own  complement.  Serum  (anti- 
bodies!), antigen,  and  complement  are  mixed  and  placed 
for  one  hour  in  the  incubator  at  37°  C.  (98.6°  F.),  so  that 
the  second  interaction  may  occur.  Then,  after  the  addition 
of  amboceptor  and  blood-corpuscles,  the  mixture  is  again 
put  for  two  hours  in  the  incubator,  so  that  if  the  second 
interaction  (from  want  of  antibodies)  has  not  occurred  (the 
complement  thus  not  having  been  used  up)  the  first  inter- 
action may  occur  (haemolysis). 

The  nature  and  significance  of  the  fixing  elements  con- 
tained in  the  serum  of  syphilitics  is  not  yet  clearly  under- 
stood, and  this  is  also  the  case  in  regard  to  the  active  ele- 
ments in  the  extract  of  the  luetic  liver.  Wassermann,  Neis- 
ser,  and  Bruck  originally  assumed  that  we  have  before  us  a 
specific  antibody-reaction.  Bruck  still  adheres  to  this  view, 
though  objections  have  been  raised  to  it  as  follows : 

(a)  A  reaction  of  equal  effect  has  been  obtained  when 
the  extract  was  made  from  normal  organs  instead  of  from 
luetic  liver.    The  luetic  liver  extract  could  also  be  replaced 
by  other  substances  which  normally  exist  in  the  organism, 
and  which  can  be  manufactured  chemically  pure. 

(b)  As  I  mentioned  in  the  introduction,  it  has  been 
proved  that  the  W.  R.  cannot  be  regarded  as  absolutely 
specific  in  the  clinical   sense.     It  has  been  occasionally 
demonstrated   in   leprosy,   malaria,   frambcesia,   recurrent 
fever,  and,  perhaps,  also  in  marked  cachexia  associated  with 
tuberculosis,  diabetes,  etc.    It  has  recently  been  asserted 
that  the  serum  of  plague  cases  also  shows  positive  W.  R. 
The  occurrence  of  this  reaction,  however,  is  not  constant  in 
these  diseases,  and  depends  greatly  on  the  quality  of  the 


BEHAVIOR  OF  THE  WASSERMANN  REACTION     345 

extract.  There  exist  extracts  which  are  excellent  to  prove 
a  previous  syphilitic  infection,  but  which  nevertheless 
yield  no  positive  reaction  with  scarlatina  serum,  while 
others  react  beautifully  both  with  the  serum  from  scarlatina 
and  from  syphilitic  patients.  In  scarlatina — the  only  dis- 
ease which  in  our  latitudes  (Germany)  need  be  considered 
in  the  differential  diagnosis — the  positive  reaction  further 
depends  on  the  disease  being  of  recent  date,  for  after  four 
weeks  at  the  most  the  serum  of  a  scarlatina  patient  loses  the 
power  of  diverting  the  complement  of  the  haemolytic  system. 
Taking  all  the  above  circumstances  into  consideration,  it  is 
hardly  admissible  to  speak  of  the  reaction  as  specific  in  the 
clinical  sense;  it  can  only  be  described  as  characteristic. 
It  is,  however,  not  intended  by  this  statement  to  restrict 
the  practical  usefulness  of  the  W.  B. 

The  Significance  of  Lymphocytosis. — What  does  the  in- 
crease of  the  lymphocytes  (pleocytosis)  in  the  cerebro- 
spinal  fluid  prove?  Pleocytosis  occurs  in  about  95  per  cent, 
of  syphilitic  and  meta syphilitic  affections  of  the  central 
nervous  system,  but  may  also  be  noted  in  other  organic 
non-syphilitic  nervous  diseases,  though  much  less  fre- 
quently, nearly  always  in  a  low  degree,  and  only  quite  excep- 
tionally with  great  intensity.  In  genuine  syphilitic,  and 
still  more  so  in  meta  syphilitic,  diseases  (general  paralysis 
and  tabes)  of  the  nervous  system,  the  lymphocytosis  is  in- 
tense, or  even  very  intense  (50  to  300,  even  to  600  and  900 
lymphocytes  in  the  field  of  vision). 

One  should  not  neglect  to  mention  that  there  are  cases 
of  tabes  in  which  lymphocytosis  is  absent,  and  especially 
is  this  apt  to  occur  in  old  stationary  cases. 

Of  great  importance  is  the  fact  first  stated  by  Merz- 
bacher,  afterwards  by  Schoenborn  and  by  Apelt,  that  in- 
dividuals who  have  once  been  infected  by  syphilis  may  show 
a  weak  or  moderate  lymphocytosis  even  in  the  absence  of 
any  clinical  symptoms  of  organic  nervous  disease.  Accord- 
ing to  our  statistics,  it  occurs  in  about  30  to  40  per  cent,  of 
the  cases.  We  may  well  conclude,  therefore,  that  the  find- 
ing of  lymphocytosis,  especially  if  it  is  of  great  intensity, 
nrast  point  with  great  or  even  very  great  probability  to  the 


346  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

existence  of  a  syphilitic  organic  nervous  disease,  but  it 
does  not  prove  it  with  certainty. 

Concerning  the  kind  of  cells  distinction  was  formerly 
made,  and  in  fact  still  is,  between  the  lymphocytes  and 
leucocytes.  The  old  technique  does  not  permit  us  to  differ- 
entiate closely  the  individual  cells  from  one  another.  Since 
the  devising  of  a  new  technique  by  Alzheimer  the  different 
types  of  cells  may  be  recognized.  Alzheimer,  and  later 
O.  Rehm,  were  able  to  differentiate  small  and  large  lympho- 
cytes, nucleated  leucocytes,  eosinophile  cells,  plasma  and 
lattice-like  cells,  and  also  macrophage  cells,  while  Szecsi 
was  able  to  differentiate  neutrophile  and  eosinophile  leuco- 
cytes, microlymphocytes  and  endothelial  and  plasma  cells. 
It  is  at  the  present  time  doubtful  whether  the  recognition 
of  these  cell  differences  will  ever  become  of  any  practical 
importance  in  the  diagnosis  of  syphilogenetic  diseases.  The 
attempt  to  recognize  in  the  spinal  fluid  a  characteristic  cell- 
picture  for  the  development  of  a  paresis,  or  to  foretell  the 
time  of  progression  of  a  syphilogenetic  affection,  has  not 
as  yet  been  crowned  with  success. 

The  Origin  of  the  Cellular  Elements. — We  do  not  know  as 
yet  the  origin  of  these  cells.  There  are  two  theories  which 
stand  in  direct  antithesis  to  one  another  to  explain  this. 
Nissl  and  Merzbacher  believe  that  the  cells  transmigrate 
from  the  blood,  while  Fischer  and  Szecsi,  in  support  of 
the  Unna-Oppenheim  view,  consider  them  of  histological 
origin,  that  is,  that  the  connective  tissue  of  the  lepto- 
meninges  is  responsible  for  their  appearance  in  the  spinal 
fluid.  This  much,  however,  is  sure:  that  pleocytosis  is  the 
result  of  an  inflammatory  irritative  condition  in  the  lepto- 
meninges.  It  has  been  demonstrated  by  such  observers 
as  Nissl,  Ranke,  Fischer,  0.  Rehm,  and  others,  by  the  micro- 
scopical examination  of  the  infiltrations  in  the  leptomen- 
inges,  that  similar  cellular  elements  are  found  there. 

The  Significance  of  Phase  I. — The  ' '  Phase  I ' '  reaction  may 
occur  in  all  organic  affections  of  the  central  nervous  sys- 
tem. Like  lymphocytosis,  it  is  also  observed  almost  without 
exception  (95  per  cent.,  and  some  authors  state  even  100  per 
cent.)  in  syphilogenetic  diseases,  both  syphilitic  and  meta- 


BEHAVIOR  OF  THE  WASSERMANN  REACTION     347 

syphilitic.  But,  whereas  lymphocytosis  occurs,  in  addition, 
in  persons  who  were  once  infected  with  syphilis,  but  do  not 
suffer  from  organic  nervous  disease,  a  Phase  I  reaction 
occurs  exclusively  in  patients  with  organic  nervous  disease ; 
it  is  never  observed  in  persons  who  have  once  been  infected 
by  syphilis  and  suffer  now  only  from  a  functional  neurosis 
(general  nervousness,  neurasthenia,  hypochondria  sis,  etc.). 
This  important  fact  enables  us  to  decide  the  diagnosis  in 
cases  which  frequently  occur  in  practice,  and  in  which  we 
have  to  face  the  question  whether  it  is  an  early  state  of 
general  paralysis  in  a  luetic  individual,  or  neurasthenia, 
or  simple  nervousness,  or  submaniacal  phases  of  a  circular 
psychosis  in  persons  who  have  previously  had  syphilis. 
In  all  such  cases  the  "Phase  I"  reaction  will  not  be  posi- 
tive so  long  as  it  is  not  a  case  of  general  paralysis.  On  the 
other  hand,  however,  the  "Phase  I"  reaction  is  unable  to 
assist  the  differential  diagnosis  between  a  non-syphilitic 
organic  cerebral  or  spinal  affection  and  a  syphilitic 
nervous  disease;  for  instance,  it  cannot  help  us  towards  a 
decision  between  multiple  sclerosis  and  cerebrospinal 
syphilis. 

The  "Phase  I"  reaction  in  organic  non-specific  affec- 
tions is  usually  slight,  often  very  slight,  in  character,  and 
sometimes  is  entirely  absent,  while  in  all  syphilogenetic 
organic  nervous  diseases  it  is  almost  always  strong,  and 
often  very  strong. 

The  Wassermann  Reaction  in  Recent  Cases  of  Syphilis  with- 
out Nervous  Symptoms. — Recently  M.  Frankel,  as  a  result 
of  studies  by  Frankel,  Heiden,  von  Zaloziesky,  and  von 
Friihwald,  conducted  from  examinations  of  the  spinal  fluid 
of  fresh  primary  and  secondary  syphilitics,  has  been  able 
by  the  utilization  of  Hauptmann's  method  to  obtain  a  posi- 
tive Wassermann  in  some  cases  (five  in  fifteen  cases),  when 
the  patients  manifested  no  symptoms  whatever  of  disease 
of  the  nervous  system. 

That  the  Wassermann  reaction  may  occur  in  the  spinal 
fluid  in  recent  cases  of  syphilis  with  nervous  symptoms  since 
the  examinations  and  reports  of  Levaditi,  Eavout,  Wechsel- 
mann,  and  others,  is  a  well-established  fact. 


348 

Also  since  the  studies  of  Ravout,  E.  Meyer,  and  others, 
it  has  been  known  that  lymphocyte  sis  in  recent  secondary 
syphilis  with  nervous  symptoms  may  occur.  M.  Frankel 
reports  likewise  the  "Phase  I"  reaction  as  occurring  in  rare 
cases  in  secondary  syphilis  where  no  nervous  symptoms  are 
present.  The  reaction  in  these  cases  was  always  weakly 
positive.  It  is  extremely  important  that  such  examinations 
should  be  more  frequently  made  and  that  the  patients  with 
positive  findings  in  the  spinal  fluid  be  kept  under  observa- 
tion. Only  in  this  way  in  time  will  we  ever  be  able  to  solve 
the  problem  as  to  whether  such  patients  are  the  ones  who 
later  on  develop  tabes,  paresis,  and  cerebrospinal  lues. 

The  Significance  of  the  Wassermann  in  the  Blood. — In  rela- 
tion to  syphilitic  organic  nervous  diseases  we  must  clearly 
understand  that  it  can  tell  us  no  more  than  that  the  individ- 
ual has  at  one  time  been  infected  by  syphilis,  but,  according 
to  the  opinion  of  most  authors,  the  reaction  is  unable  to 
indicate  whether  the  individual  still  carries  spirochaetes,  or 
whether  the  body  somewhere  still  contains  syphilitic  prod- 
ucts which  are  no  longer  manifested  clinically.  The  reaction 
is  entirely  unable  to  tell  us  whether  in  a  person  with  an 
organic  nervous  disease  the  latter  is  of  a  syphilitic  nature  or 
not.  The  W.  E.  of  the  blood,  therefore,  aids  us  practically 
only  in  so  far  as  it  enables  us  to  determine  the  former  infec- 
tion in  spite  of  a  negative  history,  or  in  the  absence  of  som- 
atic signs.  By  this  means  the  diagnosis  as  to  a  syphilitic  ner- 
vous disease  becomes  in  many  cases  much  more  probable, 
but  this  is  all  that  the  reaction  can  do.  I  may  further  state 
that  the  absence  of  the  W.  B.  in  the  blood  by  no  means 
excludes  the  possibility  of  a  syphilitic  disease.  I  need  only 
point  to  tabes  dorsalis,  in  which,  according  to  statistics 
in  all  countries,  the  reaction  is  absent  in  30  to  40  per  cent., 
i.e.,  it  is  present  only  in  60  to  70  per  cent.  There  are  also 
many  cases  of  genuine  syphilitic  disease  of  the  brain  and 
spinal  cord  where  this  reaction  is  absent.  This  absence  has 
an  evident  differential-diagnostic  value  only  in  regard  to 
general  paralysis.  I  have  published  my  investigations  in 
this  direction  in  the  jubilee  paper  to  mark  the  sixtieth  birth- 
day of  Professor  Unna.  Many  authors  have  stated  that  this 


BEHAVIOR  OF  THE  WASSERMANN  REACTION     349 

reaction  occurs  almost  without  exception  in  general 
paralysis,  certainly  in  more  than  95  per  cent,  of  the  cases. 
When  this  reaction,  therefore,  is  not  found,  we  may  justly 
doubt  the  correctness  of  the  diagnosis. 

General  Paralysis. — I  have  further  proved  that  if  one  esti- 
mates the  W.  E.  in  the  blood  (by  a  procedure  introduced 
by  Zeissler  in  the  laboratory  of  Much)  for  higher  degrees, 
one  seldom  receives  any  low  values  in  paralysis.  If  we 
establish  five  degrees  of  intensity  of  the  W.  B.  in  the  blood 
(the  weakest  being  1,  the  strongest  5),  the  degrees  5  and  4 
are  by  far  the  most  frequent  in  general  paralysis,  degree  3 
is  still  fairly  frequent,  while  degree  2  occurs  rarely,  and 
degree  1  never. 

After  having  now  briefly  explained  the  usefulness  and 
also  the  weakness  of  the  three  reactions — (a)  lymphocy- 
tosis,  (b)  "Phase  I,"  (c)  W.  E.  in  the  blood — there  re- 
mains still  the  discussion  of  the  W.  E.  in  the  cerebrospinal 
fluid. 

The  Wassermann  Reaction  in  the  Spinal  Fluid. — After  the 
W.  E.  had  been  discovered  in  tabes  and  general  paralysis 
in  the  blood,  Plant  and  Wassermann  demonstrated  it  also 
in  the  cerebrospinal  fluid.  This  had  a  great  theoretical 
significance,  for  many  authors  had  not  yet  acknowledged 
the  etiological  connection  between  syphilis  and  general 
paralysis,  and  between  tabes  and  general  paralysis.  The 
first  tests  led  to  the  opinion  that  the  W.  E.  in  the  cerebro- 
spinal fluid  of  tabetics  occurs  frequently,  but  it  was  soon 
discovered  that  it  occurs  still  more  frequently  in  general 
paralysis,  viz.,  almost  100  per  cent. 

In  1909,  at  the  Annual  Congress  of  the  American  Medi- 
cal Association  in  Atlantic  City,  I  was  able  to  state  that  the 
W.  E.  in  the  cerebrospinal  fluid  of  tabetics  is  relatively  rare, 
for  it  occurs  perhaps  only  in  10  to  20  per  cent,  of  the  cases. 
Already,  in  1908,  Plant,  Stertz,  and  myself  had  found  that 
the  W.  E.  in  the  cerebrospinal  fluid  is  rare  in  cases  of 
cerebrospinal  syphilis.  Before  it  was  known  that  this  reac- 
tion is  infrequent  in  tabetics,  the  presence  or  absence  of  it 
had  been  used  for  differential  diagnosis  between  tabes  and 
spinal  syphilis ;  but  this  is  now  no  longer  possible.  These 


350  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

new  methods  of  examination  still  left  undecided  those  cases 
in  which  the  clinical  symptoms  had  also  failed  to  assist  the 
differential  diagnosis  with  certainty.  I  refer  to  cases  of 
multiple  sclerosis,  tumor  of  the  brain  or  of  the  spinal  cord, 
as  against  cases  of  cerebral,  spinal,  or  cerebrospinal  syph- 
ilis. It  is  well  known  how  similar  these  clinical  pictures 
often  are,  and  that  the  difficulty  of  differential  diagnosis 
becomes  even  greater,  if  the  individual  in  whom  one  sus- 
pects multiple  sclerosis,  or  cerebral  or  spinal  tumor,  has 
passed  through  a  syphilitic  infection.  In  such  cases  the 
method  of  making  a  quantitative  "W.  R.  in  the  cerebrospinal 
fluid  was  of  assistance.  This  method  had  first  been  applied 
by  Hauptmann  and  Hoessli  in  Much's  laboratory  at  the 
Eppendorf  Hospital. 

With  this  method,  by  the  use  of  larger  quantities  of 
cerebrospinal  fluid,  it  is  possible  to  prove  the  W.  R.  in  such 
syphilitic  nervous  diseases  as  tabes,  and  cerebral,  spinal,  or 
cerebrospinal  syphilis,  while,  on  the  other  hand,  cases  of 
multiple  sclerosis,  cerebral  and  spinal  tumors  (extra-  and 
intramedullary)  never  show  the  reaction,  even  if  one  in- 
creases the  quantity  of  cerebrospinal  fluid  from  the  original 
0.2  c.c.  of  Wassermannto  1.0  c.c.  and  more.  I  have  reported 
from  my  own  practice  several  examples  to  show  how  I  suc- 
ceeded in  diagnosing  correctly  a  non-syphilitic  spinal  dis- 
ease in  a  person  who  had  been  formerly  infected  by  syphilis, 
and  who  suffered  from  acute  spinal  paraplegia.  The 
autopsy  revealed  an  intramedullary  soft  gliosarcoma  of  the 
spinal  cord,  as  well  as  genuine  luetic  gummata  of  the  lungs. 
In  another  case  I  was  able  to  diagnose  in  a  former  luetic 
person  a  non-luetic  tumor  pressing  on  the  optic  nerves, 
and  the  autopsy  revealed  a  large  arteriosclerotic  aneurism 
of  the  anterior  communicating  artery.  In  a  third  case, 
with  a  clinical  history  of  syphilis,  I  could  predict  the  syph- 
ilitic origin  of  a  tumor  with  localizing  symptoms  in  the  left 
frontal  lobe,  and  the  autopsy  revealed  a  tough  gumma  in  the 
left  frontal  lobe.  In  a  fourth  case  I  was  able  to  diagnose 
in  a  patient  with  a  history  of  a  previous  specific  infection 
that  a  non-specific  tumor  of  the  left  anterior  central  convo- 
lution was  the  cause  of  the  clinical  picture  presented,  and 


BEHAVIOR  OF  THE  WASSERMANN  REACTION     351 

the  operation  which  led  to  complete  recovery  confirmed  this 
diagnosis. 

I  mention  these  four  cases  from  a  large  number  of  the 
same  category  in  order  to  show  that  our  methods  have 
proved  useful  in  practice.  The  examination  of  the  cerebro- 
spinal  fluid  for  W.  R.,  with  and  without  estimation  of  quan- 
tity, should  be  used  especially  in  cases  where  it  is  important 
to  find  out  whether  or  not  the  organic  nervous  disease  is  of 
a  syphilitic  nature. 

The  Value  of  the  Four  Reactions  in  Differential  Diagnosis. — 
The  results  of  the  four  reactions  are  of  especial  value  in 
differentiating  organic  disease  of  the  nervous  system  as  a 
consequence  of  alcoholism,  particularly  where  syphilis  is 
found  in  the  anamnesis.  It  is  well  known  how  much  pseudo- 
tabes  alcoholica  and  pseudoparesis  alcoholica  can  resemble 
the  syphilogenetic  tabes  and  paresis.  In  the  alcoholic 
peripheral  neuritis  and  myelitis  funicularis,  as  well  as  the 
alcoholic  paresis,  lymphocytosis,  Phase  I,  and  the  Wasser- 
mann  reaction  in  the  spinal  fluid  are  absent,  while  in  those 
cases  where  the  patient  had  a  previous  syphilitic  infection 
the  W.  R.  in  the  blood  may  be  positive. 

I  deem  it  practical  to  sum  up,  in  conclusion,  the  use  of 
the  "four  reactions,"  in  tabulated  form,  as  follows: 

1.  Examination  of  the  Blood.     Wassermann  Reaction. 

(a)  Positive:  It  is  characteristic  of  syphilis;  there  are 
few,  if  any,  exceptions.     The  reaction  is  positive;  also  in 
some  cases  of  recent  scarlatina,  malaria,  leprosy,  fram- 
boesia,  etc. 

Positive  W.  R.  of  the  blood  means  no  more  than  that  the 
person  has  once  been  in  contact  with  syphilis  (hereditary 
or  acquired),  or  that  somewhere  in  the  body  a  specific  lesion 
still  exists,  but  it  does  not  mean  necessarily  that  the  disease 
in  question  must  be  luetic. 

(b)  Negative:  It  can  be  used  in  differential  diagnosis 
with  great  probability  against  the  existence   of  general 
paralysis,  for  the  blood  of  paralytics  reacts,  with  rare  ex- 
ceptions, positive. 

2.  Examination  of  the  Cerebrospinal  Fluid. 

(a)  Normal  fluid:  Pressure  in  vertical  tube  90  to  130 


352  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

mm.  water ;  Phase  I  reaction  negative ;  5  to  6  cells  per  c.mm. 
at  the  most  (Fuchs-Bosenthal) ;  "W.  E.  negative  after  the 
original  method  (0.2  c.c.  of  fluid),  as  well  as  after  using 
larger  quantities  (0.3  c.c.  to  1.0  c.c.  of  the  fluid). 
(b)  Pathological  fluid : 

(1)  Increased  pressure  of  escaping  fluid  (above  150  mm. 
water). 

(2)  Phase  I  reaction  positive. 

(3)  Lymphocytosis. 

These  three  symptoms  (either  combined  or  singly)  indi- 
cate that  there  exists  an  organic  disease  of  the  central  ner- 
vous system  (specific  or  non-specific).  If  Phase  I  and 
lymphocytosis  are  positive,  these  two  reactions  indicate  the 
probability  of  syphilis. 

(4)  The  W.  R.  of  the  cerebrospinal  fluid  decides  whether 
the  disease  of  the  central  nervous  system  is  of  a  luetic 
nature.     If  it  is  already  positive  by  the  original  method 
(0.2  c.c.  of  fluid)  there  is  great  probability  that  the  case  in 
question  is  one  of  general  paralysis  or  taboparalysis ;  less 
frequently,  of  tabes  and  of  cerebrospinal  syphilis.    In  the 
large  majority  of  cases  of  general  paralysis  the  W.  B.  is 
usually  positive  on  using  only  0.2  c.c.  of  fluid,  but  in  some 
few  cases,  and  in  most  cases  of  cerebrospinal  syphilis  and  of 
tabes,  the  W.  B.  is  positive  only  on  using  larger  quantities 
of  cerebrospinal  fluid  (0.3,  0.4  up  to  1.0  c.c.). 

Typical  Findings. — 1.  General  Paralysis  or  Tabopa- 
ralysis. (1)  W.  B.  in  the  blood  positive  (almost  100  per 
cent.). 

Pressure  of  cerebrospinal  fluid  often  increased.  (2) 
Phase  I — reaction  positive  (in  about  95  to  100  per  cent.), 
usually  strongly  positive.  (3)  Lymphocytosis  (in  about  95 
per  cent.),  usually  marked.  (4)  W.  B.  in  the  cerebrospinal 
fluid. 

(a)  Positive  in  about  90  per  cent,  after  the  original 
method  (0.2  c.c.  of  fluid). 

( b )  In  100  per  cent,  by  using  larger  quantities. 
2.  Tabes  (without  combination  with  paralysis). 

(1)  W.  B.  in  blood-serum  positive  in  about  70  per  cent. 
Pressure  of  cerebrospinal  fluid  often  increased. 


BEHAVIOR  OF  THE  WASSERMANN  REACTION     353 

(2)  Phase  I — reaction  positive  in  about  95  per  cent., 
usually  strong. 

(3)  Lymphocytosis  in  about  90  to  95  per  cent.,  usually 
strong. 

(4)  W.  R.  in  the  cerebrospinal  fluid. 

(a)  After  original  method  (0.2  c.c.)  positive  in  about 
20  per  cent. 

(b)  With  larger  quantities  positive  in  almost  100  per 
cent. 

3.  Cerebrospinal  Lues. 

(1)  W.  R.  in  blood-serum  positive  in  about  70  to  80 
per    cent.     Pressure    of    cerebrospinal    fluid    frequently 
increased. 

(2)  Phase  I  reaction,  only  in  exceptional  cases,  negative, 
otherwise  positive,  generally  not  as  strong  as  in  paresis 
and  tabes. 

(3)  Lymphocytosis,  like  Phase  I,  almost  always  positive, 
but  not  so  marked  as  in  paresis  and  tabes. 

(4)  W.  R.  in  the  cerebrospinal  fluid. 

(a)  With  original  method   (0.2  c.c.)   positive  in  about 
20  per  cent. 

(b)  With  larger  quantities  of  fluid  almost  always  posi- 
tive   (of   special   value   for  the  differential   diagnosis  as 
against  multiple  sclerosis). 

The  Importance  of  the  Four  Reactions  in  Determining  the 
Recovery  of  the  Patient. — The  testing  of  the  four  reactions 
is  also  exceedingly  important  in  those  cases  where  the  ques- 
tion is  to  be  determined  as  to  whether  complete  recovery  has 
occurred.  Only  where  all  four  reactions  are  negative  are 
we  justified  in  speaking  of  an  objective  cure.  The  old  say- 
ing of  Fournier,  "Syphilis  does  not  die,  it  only  sleeps," 
may  be  applied  with  correctness  to  the  majority  of  cases  of 
cerebrospinal  syphilis  and  almost  all  cases  of  tabes  and 
paresis.  Where,  however,  the  four  reactions  are  all  nega- 
tive one  may  conclude  that  the  syphilis  of  the  patient  has 
really  ceased  to  exist.  I  have  observed  a  large  number  of 
such  cases.  The  persistence  of  one  or  more  of  the  four 
reactions  demands  a  continuation  of  the  treatment  in  cere- 
brospinal lues.  In  tabes  and  paresis  the  persistence  of  the 

23 


354  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

three  reactions  in  the  spinal  fluid  is  no  indication  for  the 
continuance  of  the  treatment,  for  in  these  two  diseases  the 
three  reactions,  except  in  rare  cases,  are  uninfluenced  by 
any  therapy  that  we  are  at  present  familiar  with. 

The  Significance  of  Haemolysin. — Recently  investigations 
have  been  instituted  by  Von  Weil  and  Kafka  concerning 
the  hsemolysin  present  in  the  spinal  fluid.  Hauptmann,  in 
my  department  at  Eppendorf,  and  Eichelberg,  in  Cramer's 
clinic  at  Gottingen,  are  at  present  making  control  examina- 
tions. Whether  this  reaction  as  a  matter  of  fact  occurs 
only  in  paresis,  as  Von  Weil  and  Kafka  assert,  control  ex- 
aminations must  determine.  These  two  observers  them- 
selves admit  that  it  does  not  occur  in  all  cases  of  paresis. 

The  Four  Reactions  Do  Not  Solve  All  the  Questions  of 
Diagnosis. — A  closer  study  of  the  above  tabulated  summary 
shows  that  the  use  of  even  all  four  reactions  does  not  yet 
solve  all  the  questions  as  to  the  diagnosis  of  the  diseases  of 
the  central  nervous  system.  It  is  even  possible  that  the 
result  of  the  serological,  cytological,  and  chemical  examina- 
tion of  blood  and  cerebrospinal  fluid  may  be  misleading. 
For  instance,  it  happens  in  some  few  cases  that  the  blood 
of  a  general  paralytic  shows  negative  W.  B.,  and  that  in  a 
tabetic  all  four  reactions  are  negative.  This  seems  most 
likely  to  occur  in  chronic,  long-standing  cases,  and  in  heredi- 
tary tabes,  as  Plant,  Stertz,  and  I  have  shown.  It  should 
be  mentioned,  however,  that  recently  Klienburger  has  found 
all  four  reactions  positive  in  hereditary  paresis. 

The  Absence  of  the  Wassermann  Reaction  in  Syphilitic 
Arteritis. — While  it  has  never  occurred  in  the  Nonne  ward 
that  the  W.  R.  in  the  cerebrospinal  fluid  of  a  paralytic  was 
negative  by  using  larger  quantities  of  fluid,  cases  of  cere- 
brospinal syphilitic  arteritis  have  repeatedly  shown  a  total 
absence  of  fixing  elements  in  the  cerebrospinal  fluid. 

The  Importance  of  a  Thorough  Clinical  Examination. — I 
need  not  discuss  again  the  possibility  of  a  combination  of  a 
non-specific  disease  of  the  central  nervous  system  with  a 
specific  one,  whether  stated  in  the  history  or  not.  Owing 
to  the  stated  sources  of  error,  of  which  there  still  exist 
many,  it  follows  that  even  if  one  uses  all  four  reactions, 


BEHAVIOR  OF  THE  WASSERMANN  REACTION     355 

one  has  still  to  observe  the  greatest  critical  caution  in  diag- 
nosis. If  the  observer  does  not  wish  to  run  the  risk  of  grave 
diagnostic  errors,  he  has  still  to  apply  a  careful  and  detailed 
clinical  examination  in  each  individual  case,  working  hand 
in  hand  with  the  more  modern  methods.  Some  skeptics  and 
critics  have  already  sarcastically  remarked  that  the  pivot 
of  medical  examination  lies,  nowadays,  in  the  laboratory, 
but  this  is  by  no  means  correct.  It  is  noteworthy  that 
Hoche,  the  psychiater  in  Freiburg,  who  formerly  was  ex- 
ceedingly skeptical,  has  recently  in  a  monograph  concern- 
ing paresis  recognized  the  diagnostic  value  of  the  four 
reactions  after  he  himself  had  put  them  to  practical  test. 
On  the  other  hand,  we  may  say,  after  some  years  of  careful 
and  conscientious  testing  of  these  modern  methods  of  exam- 
ination, that  by  their  complete  mastery  and  their  logical 
and  critical  application,  we  have  obtained  an  excellent  aid 
towards  the  diagnosis  of  the  affections  of  the  central  ner- 
vous system,  especially  of  the  syphilitic  ones. 


XVIII 
PROPHYLAXIS 

IN  the  discussion  of  the  treatment  of  syphilis  naturally 
the  prophylaxis  comes  first.  Prophylaxis,  of  course,  con- 
sists in  the  avoidance  of  specific  infections.  When  Growers 
in  impressive  words  lays  on  the  heart  of  his  hearers  the 
importance  of  an  unbroken  chastity,  so  it  is  to  be  hoped 
that  such  advice  might  be  given  oftener  in  our  universities 
to  the  embryo  physicians.  I  am  convinced  that  many  of  our 
medical  men  do  not  properly  appreciate  their  responsibility 
when,  for  any  reason  whatever,  they  advise  sexual  inter- 
course for  their  unmarried  patients.  Nevertheless,  we  must 
all  acknowledge  that  sexual  abstinence  for  the  great  major- 
ity of  young  men  will  probably  remain  an  unattained  ideal. 
The  society  for  fighting  the  spread  of  venereal  diseases, 
which  was  founded  several  years  ago  in  Berlin  and  at  whose 
head  were  such  men  as  Neisser,  Lesser  and  others,  sought 
to  exert  a  beneficial  influence  along  educational  lines 
through  the  distribution  of  suitable  literature  and  public 
addresses.  A  similar  plan  has  been  followed  in  the  schools 
where  the  pupils  during  their  last  years  have  been  in- 
structed on  this  subject.  Of  the  school  instruction  I  do 
not  approve.  A  conscientious  father  is  the  proper  one  to 
assume  this  task,  or  possibly  the  family  physician.  At  any 
rate,  in  my  opinion,  the  sexual  theme  is  scarcely  a  fitting 
subject  to  discuss  before  a  forum  of  youths  who  are  barely 
in  their  teens.  One  can  say  at  least  to  the  growing  youth 
that  sexual  intercourse  is  not  absolutely  essential  for  his 
physical  welfare,  that  wet  dreams  furnish  the  natural  relief 
for  his  sexual  demands.  The  Catholic  clergy  demonstrate 
the  harmlessness  of  sexual  abstinence. 

With  reference  to  the  extragenital  infection  there  is  no 
prophylaxis,  because  in  the  great  majority  of  the  cases  the 
infection  takes  place  when  it  is  the  least  suspected. 

Physical  and  Psychic  Trauma,  Alcoholism. — It  belongs  to 
the  realm  of  prophylaxis  also  that  syphilitics  who  are 

356 


PROPHYLAXIS  357 

inclined  to  nervous  affections,  either  on  account  of  family 
tendency  or  through  their  individuality,  should  avoid  on  the 
physical  side  spinal  and  head  injuries,  and  on  the  psychic, 
mental  strain  and  strong  emotion.  These  recommendations, 
however,  have  only  academic  worth.  It  is  of  much  more 
practical  value  to  advise  one 's  patients  to  be  extremely  mod- 
erate in  the  use  of  alcohol  and  tobacco.  There  is  no  doubt 
that,  next  to  syphilis,  alcohol  is  the  most  injurious  nerve 
poison. 

Intelligent  Treatment  of  the  Primary  and  Secondary  Lesions. 
— Can  intelligent  and  energetic  treatment  of  the  primary 
and  secondary  lesions  banish  the  spectre  of  nervous  syph- 
ilis ?  This  question  already  has  been  thoroughly  discussed 
in  the  consideration  of  the  prognosis  of  brain  lues.  The 
answer  of  such  observers  as  Moller,  Goldflam,  Hoppe, 
Savard,  Julliard,  Schultze,  Schmaus,  and  many  others,  is 
that  one,  at  any  rate  for  many  cases,  must  reply  to  the 
above  question  in  the  negative.  I  have  heard  this  negation 
in  many  conversations  with  exceedingly  well-informed  phy- 
sicians, and  I  must  confess  that  mine  must  be  enrolled  with 
the  rest.  It  may  be  said  that  in  this  regard  the  syphilologist 
overestimates  the  importance  of  specific  treatment.  It  is 
true  for  both  brain  and  spinal  cord  what  Fournier  first 
stated  in  respect  to  the  brain :  that  there  are  cases  of  brain 
syphilis  in  which  the  tendency  of  the  toxin  from  the  very 
beginning  seems  directed  towards  the  central  nervous  sys- 
tem, and  in  a  malignant  manner,  since  antispecific  treatment 
as  a  prophylactic  fails  in  stopping  the  advance  of  the  affec- 
tion. In  spite  of  this  fact,  however,  in  every  case  it  is 
advisable,  as  early  as  possible,  to  institute  a  thorough  and 
energetic  treatment  in  primary  and  secondary  syphilis  in 
the  hope  of  warding  off  future  nervous  complications. 

It  is  worthy  of  recommendation,  if  it  could  be  carried 
out  in  general  practice,  to  advise  following  the  custom  of 
the  St.  George  General  Hospital  in  Hamburg.  This  hos- 
pital gave  to  every  syphilitic  patient  at  the  time  of  their 
discharge  the  following  printed  instructions: 

You  are  affected  with  a  venereal  disease  (syphilis).  You  must  take 
care  that  you  do  not  transmit  your  disease  to  others,  which  may  be  done 


358  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

by  sexual  intercourse,  kissing,  by  sleeping  in  the  same  bed  and  using  the 
same  dishes  and  drinking  out  of  the  same  cups  with  others. 

Your  disease  cannot  be  cured  in  a  -single  course  of  treatment.  You  will 
in  all  probability  again  notice  symptoms  of  your  affection,  as  ulcerated 
patches  or  pains  in  the  mouth  or  in  the  neck,  or  on  the  private  parts,  or  in 
the  form  of  an  eruption  on  the  body.  As  soon  as  you  observe  any  of  these 
symptoms  you  must  immediately  place  yourself  under  medical  care.  Beware 
of  quacks,  cures  by  nature's  methods,  and  all  such  silliness.  For  the  proper 
treatment  of  your  disease  a  thorough  knowledge  of  all  methods  of  therapy 
is  essential  and  such  knowledge  is  only  possessed  by  a  regularly  licensed 
physician.  Also  when  no  symptoms  of  your  disease  are  manifest,  you  should 
put  yourself  under  medical  care  once  every  four  months  in  order  to  take  a 
course  of  treatment.  This  course  of  treatment  does  not  necessarily  require 
your  going  to  a  hospital  or  the  giving  up  of  your  regular  work.  When  you 
have  taken  regular  courses  of  treatment  over  a  period  of  three  years,  you 
may  in  all  probability  expect  to  be  spared  the  later  and  severe  symptoms 
of  your  disease,  such  as  bone  decay,  nerve  and  spinal-cord  affections,  prema- 
ture apoplexy,  etc. 

Only  after  four  or  five  years  has  elapsed  since  your  infection,  and  then 
after  securing  the  approval  of  your  physician,  should  you  think  of  getting 
married.  If  you  do  not  heed  this  injunction,  you  will  be  liable  to  transmit 
your  disease  to  your  wife  and  children. 

By  suitable  and  long-continued  treatment  you  can  expect  to  obtain  a 
complete  cure. 

Treatment  with  Mercury  and  lodid. — In  lues  of  the  brain 
and  spinal  cord  the  treatment  should  begin  as  soon  as  the 
diagnosis  is  made.  The  warning  of  Althaus  against  the 
administration  of  iodid  in  the  very  beginning  of  brain 
syphilis  does  not  seem  justified. 

Inunctions,  Injections,  Sack-therapy  and  Internal  Medication. 
—When  it  is  possible  to  carry  out,  insist  upon  treatment  by 
mercurial  inunctions,  because  it  is  the  most  thorough  and 
quickest  in  effect.  Neumann,  of  Vienna,  after  an  unusually 
large  experience,  has  also  come  to  this  conclusion.  As  a 
supplementary  treatment  when  the  symptoms  do  not  dis- 
appear quickly  enough  or  remain  unchanged  in  the  first 
three  to  six  weeks,  every  four  or  five  days  an  injection  into 
the  nates  may  be  made  of  salicylate  of  mercury  in  a  10  per 
cent,  solution,  of  which  the  following  is  the  formula : 

Hydrarg.  salicyl 1.0 

Paraffin  liquid    10.0 

Sig. — To  be  well  shaken  before  using. 

Lewin's  Sublimate  Injection. — This  injection  is  convenient 
in  those  cases  where,  for  some  reason,  such  as  lack  of 


PROPHYLAXIS  359 

time,  concealment,  skin  disease  before  the  beginning  of  the 
inunctions  or  as  a  consequence  of  them,  so  that  the  rubbings 
cannot  be  administered  and  the  physician  considers  neces- 
sary a  more  frequent  dose  of  mercury  than  can  be  given 
of  either  the  mercury  salicylate,  oleum  cinereum,  or  calomel. 

Formula:  Lewin's  sublimate  injection. 

Hydrarg.  bichlor.  corros 0.3 

Sodium  chloride 3.0 

Aqua  destillata    30.0 

Sig. — 1   c.cm.   injected  equally   in  two  different  places  in 
each  buttock,  once  or  twice  daily. 

Welander  has  recommended  the  mercurial  sack-therapy. 
In  this  method  of  mercurial  administration  the  patient 
wears  a  flannel  cloth  smeared  with  mercury  or  a  flannel 
sack  filled  with  it  around  his  body.  Welander  states  that 
this  form  of  mercurial  treatment  works  quickly  and  effec- 
tually. The  apron  or  sack  should  be  worn  without  inter- 
ruption for  from  four  to  six  weeks  and  acts  upon  the 
organism  by  slow  evaporation.  This  method  has  been  en- 
dorsed by  such  men  as  Schuster,  Neumann,  and  A.  Neisser. 
It  is  an  agreeable  way  of  giving  mercury,  but  not  so  certain 
as  the  inunction  and  injection  methods.  The  injections  of 
calomel  and  oleum  cinereum  bring  with  them  the  danger 
of  abscess  formation.  Notwithstanding  this,  Harthing  and 
Dreyfus  have  recently  strongly  recommended  calomel 
injections. 

The  following  is  the  formula  for  a  calomel  injection : 

Calomel 

Sodium  chloride  aa  5.0 

Aqua  destillata    50.00 

Mucilage  of  tragacanth    2.5 

For  an  oleum  cinereum  injection : 

Hydrarg. 

Lanolin  aa   3.0 

Olive  oil    4.0 

The  dose  of  both  of  these  formulas  is  1  c.cm.  injected  once 
every  fourth,  fifth  or  sixth  day.  Five  or  six  injections  are 
equal  to  a  course  of  from  24  to  30  inunctions. 


360  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

Recently  Zierler  in  Neisser's  clinic  has  recommended 
quite  highly  injections  of  gray  oil.  He  uses  a  mixture  which 
consists  of  40  per  cent,  mercury,  13.5  lanolin  and  46.5 
per  cent,  paraffin,  and  which  remains  liquid  at  15  de- 
grees Celsius;  that  is,  does  not  need  to  be  warmed  but 
only  to  be  shaken  before  using.  This  solution  causes  the 
least  pain.  The  dose  is  0.28  cm.  Zierler  regards  this  mix- 
ture when  properly  prepared  as  absolutely  free  from  dan- 
ger and  ascribes  to  it  the  best  results  in  secondary  and 
tertiary  lues. 

Neisser  recommends  the  gluteal  region  as  the  best  place 
for  injections.  It  is  important  to  remember  when  adminis- 
tering mercury  by  injection  that  iodid  should  never  be 
given  at  the  same  time,  otherwise  the  formation  of  gluteal 
gummata  may  occur. 

In  France  and  England  mercury  is  administered  more  in 
the  form  of  pills  and  powders  than  in  Germany.  Since  the 
results  obtained  cannot  be  doubted,  when  one  is  unable  to 
give  inunctions  or  injections  the  mercury  may  be  adminis- 
tered in  this  way.  If  diarrhoea  appears  as  a  consequence 
of  the  pill  or  powder  medication  then  sufficient  opium  may 
be  added  to  relieve  it. 

Ricord's  pills,   hydrargyri,  iodidum  flavum. 

Lectucarni  gallici  aa    3.0 

Ext.   opii   aquae    1.0 

Ext.  conii  maculati   6.0 

in  pill   No.  60 

Sig. — Take  one   pill    15   minutes   after   the  evening  meal. 
Later,  one  pill  morning  and  evening. 

The  following  is  the  formula  for  hydrargyri  tannicum : 

Hydrarg.  oxydulat.  tann 5.0-10.0 

Bol.   alb.    £        gat    ad  NQ     1Q() 

Glycerin    )    ^ 

Sig. — Take  two  pills  daily,  morning  and  evening. 

Recently  syphilologists  have  used  a  preparation  called 
Enesol.  It  is  dispensed  in  ampules,  each  2  c.cm.  or  ampule 
containing  .06  gramme  Enesol.  It  is  given  either  in  twenty 
injections  in  succession  or  in  ten  injections  with  a  pause. 
This  preparation  sometimes  causes  colitis. 


PROPHYLAXIS  361 

Do  not  be  timid  in  your  doses,  but  begin  at  once  with 
4  Gm.  unguentum  ciner.  as  an  inunction  and  gradually  in- 
crease up  to  5  and  6  Gm.  My  former  chief,  Dr.  Engel 
Reimers,  who  has  had  an  unusually  large  experience,  came 
to  the  conclusion  that  nothing  more  was  to  be  accomplished 
by  rubbing  in  a  larger  quantity  than  6.0  Gm.  unguentum 
ciner.  at  one  time.  If  one  is  justified  in  giving  such  enor- 
mous doses  as  Krauss  has  done,  iy3  grammes  sublimate 
intramuscularly  daily  for  ten  days  in  succession,  I  doubt 
very  much. 

Mercurial  Neuritis. — The  occurrence  of  a  mercurial  poly- 
neuritis  has  been  often  doubted.  The  observations  of  Ketly, 
Forestier,  Leyden,  Spillman,  and  Etienne,  according  to  my 
opinion,  leave  no  foundation  for  doubt.  We  can  state  at  the 
present  time  that  the  occurrence  of  a  mercurial  polyneuritis 
must  be  admitted.  However,  such  cases  are  exceedingly 
rare,  and  in  my  experience  of  nineteen  years  in  hospital 
and  private  practice  I  have  never  seen  a  case  originating 
from  mercury  administered  as  a  therapeutic  agent.  Erb's 
experience  has  been  the  same. 

Treatment  by  Inunctions. — Whether  a  bath  should  be 
given  daily  or  only  at  the  end  of  the  inunction  cycle  depends 
upon  the  case.  The  effect  of  the  mercury  remaining  on  the 
body  several  days  at  a  time  is  more  intense  and  permanent. 
Recently  Hoesslin  has  reported  very  good  results  in  brain 
syphilis  with  the  inunction  treatment  and  daily  baths.  He 
considers  the  rule  to  bathe  only  after  a  cycle  is  ended  not 
well  grounded.  After  the  termination  of  a  cycle,  whether  it 
is  of  four,  five,  or  six  days'  duration,  to  allow  two  days  of 
intermission  (the  first  of  which  should  be  used  for  cleansing 
purposes  with  soap  and  lukewarm  water)  is  to  be  strongly 
recommended. 

In  regard  to  the  number  of  inunctions,  my  custom  in  all 
cases,  even  though  the  symptoms  have  disappeared,  is  to 
administer  about  thirty. 

In  every  case  of  lues  of  the  nervous  system  iodid  should 
also  be  given,  for  it  is  regarded,  and  correctly,  as  a  specific 
in  the  tertiary  syphilitic  manifestations  and  the  specific 
pathological  processes  which  form  the  foundation  of  ner- 


362  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

vous  syphilis  are  at  the  present  time,  in  general,  enrolled 
in  this  category.  The  period  of  time  between  the  invasion 
of  lues  in  the  body  and  the  appearance  of  inflammatory  and 
gummatous  products  according  to  this  classification  is  not 
material. 

It  is  best  to  begin  with  small  doses,  iy2  grammes  pro 
die.  If  the  iodid  is  tolerated  well,  then  increase  rapidly 
to  larger  doses.  One  may  give  without  harm  for  two  or 
three  months  at  a  time  from  4  to  6  grammes  of  iodid  daily. 
In  many  cases  sodium  iodid  is  tolerated  better  than  potas- 
sium or  strontium,  and  rubidium  iodid  better  than  the 
sodium.  A  combination  of  different  iodid  salts  has  been 
much  recommended,  as  of  the  potassium,  sodium,  and 
ammonium.  The  iodid  is  often  better  borne  if  one  adds 
to  one  teaspoonful  of  the  customary  dose  of  iodid  two  or 
three  drops  of  Fowler 's  solution.  It  is  not  advisable  to  stop 
the  iodid  at  the  first  appearance  of  intoxication  symptoms, 
since  very  often  after  a  short  time  the  patient  becomes 
accustomed  to  the  drug  and  these  pass  away. 

Among  the  newer  preparations  of  iodine  iodipin  is  to 
be  recommended.  Iodipin  comes  in  10  or  25  per  cent,  solu- 
tions of  iodine  in  oil  of  sesame.  It  may  be  injected  into  the 
gluteal  region  in  doses  of  from  10  to  20  c.cm.  daily  for  a 
period  of  10  days,  then  an  interval  and  a  repetition  of 
the  injections.  Internally  iodipin  may  be  given  in  the 
10  per  cent,  solution  in  teaspoonful  doses  three  times  a  day. 
Another  preparation  of  iodine  that  is  comparatively  free 
from  bad  effects  is  sajodin.  It  contains  60  per  cent,  of 
iodine.  The  dose  is  from  1  to  3  grammes  pro  die,  which  may 
be  increased  even  to  6  grammes.  A  large  number  of  other 
iodine  preparations  have  recently  made  their  appearance, 
the  most  of  which  are  unimportant. 

Indications  for  Mercury  and  Iodid. — Both  of  these  thera- 
peutic agents,  mercury  and  iodine,  are  able  to  influence 
syphilitic  tissue.  In  general  the  impression  prevails  at  the 
present  time  that  mercury  has  the  most  effect  on  a  true  in- 
flammation, while  iodine  acts  more  efficiently  on  gummatous 
formations. 

In  a  study  of  the  subject  by  Stern  made  from  Jadas- 


PROPHYLAXIS  363 

sohn's  clinic,  and  also  from  a  review  of  the  literature,  it 
appears  that  there  are  still  widely  different  opinions  as  to 
the  relative  value  of  both  these  agents  in  the  different 
periods  and  various  lesions  of  syphilis. 

The  following  views  were  found  to  have  been  expressed 
in  the  literature  on  this  subject: 

1.  Mercury  and  iodine  are  effective  in  all  the  diseased 
processes  of  syphilis,  but  the  mercury  is  the  more  efficient 
in  the  early  stages  and  the  iodine  acts  more  energetically 
in  the  later  stages. 

2.  Mercury  acts  only  or  only  essentially  upon  the  early 
processes,  while  iodine  acts  only  or  only  essentially  upon 
the  late  processes.    At  the  same  time,  however,  it  was  quite 
generally  admitted  that  iodine  possesses  an  influence  upon 
certain  symptoms  occurring  in  the  early  period,  such  as 
occipital  headache. 

3.  Mercury  acts   on  all  the   stages   of  syphilis,   while 
iodine  chiefly  affects  the  tertiary  stage. 

The  majority  of  the  modern  authorities  take  the  view 
that  mercury  is  the  most  effectual  early  in  the  course  of  the 
disease  and  iodine  in  the  later  manifestations.  In  practice, 
however,  a  combination  of  the  two  specifics  is  usually  ad- 
ministered at  the  same  time,  since  the  differential  diagnosis 
between  early  and  late  syphilis  on  the  skin,  mucous  mem- 
brane and  internal  organs  is  often  exceedingly  difficult. 

Stern,  from  his  experience  in  Jadassohn's  clinic,  came 
to  the  conclusion  that  a  syphilis  which  had  been  treated 
in  the  earlier  stages  with  mercury,  in  the  later  stages  re- 
sponded promptly  to  iodine. 

Oppenheim  gives  the  preference  to  mercury  in  syphilis 
of  the  nervous  system. 

Action  of  Mercury  in  Non-specific  Affections. — It  may  be 
said  that  iodine  sometimes,  although  rarely,  acts  upon 
other  growths,  and  especially  gliomata,  and  Gowers  states 
that  he  saw  an  optic  neuritis,  for  the  assumption  of  whose 
specific  nature  there  was  no  other  basis  than  that  it  disap- 
peared under  the  administration  of  iodine. 

There  are  also  cases  which  present  the  clinical  picture 
of  a  cerebral  tumor  with  choked  disc  and  hemiplegia,  which 


364  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

are  not  specific  in  character  but  which  disappear  under 
inunctions  of  mercury  with  and  without  the  combination  of 
iodine.  Such  cases  in  adults  I  have  classified  under  the 
name  of  pseudotumor  cerebri.  In  eight  such  cases  in  my 
own  experience  a  partial  recovery  occurred.  Further  com- 
munications in  the  literature  on  this  subject  have  been  made 
by  Henneberg,  Berlin;  Saenger,  Hamburg;  Hoppe,  Cincin- 
nati; Finkelburg,  Bonn,  and  others.  The  occasional  in- 
fluence of  iodine  on  non-specific  tumors  has  been  acknowl- 
edged by  Oppenheim  and  Bruns  in  their  work  on  tumors. 

Duration  of  Specific  Treatment  Where  the  Symptoms  Con- 
tinue Refractory. — Concerning  the  question  as  to  how  long 
mercury  and  iodine  should  be  administered  where  no  im- 
provement in  the  symptoms  occur  opinions  differ  greatly. 
There  are  no  doubt  numerous  cases  reported  in  the  litera- 
ture in  which  the  antispecific  therapy  has  been  continued 
for  years  and  only  after  years  have  the  symptoms  disap- 
peared. Such  cases  cause  the  critical  observer  to  wonder 
whether  a  spontaneous  recovery  has  not  taken  place  during 
the  treatment  rather  than  on  account  of  it. 

In  general  one  can  state  that  in  true  cases  of  syphilis 
of  the  nervous  system  one  should  expect  an  improvement 
in  the  symptoms  at  the  end  of  the  second  week,  or  at  any 
rate  at  the  end  of  the  third  week. 

Gowers  has  expressed  the  opinion  that  what  has  not 
disappeared  after  from  six  to  ten  weeks  of  mercury  and 
iodine  administration  will  resist  further  antispecific  ther- 
apy; also  that  the  persistence  of  the  symptoms  must  not 
be  an  indication  for  the  continuation  of  the  antispecific 
treatment.  In  my  own  practice,  when  after  the  antispecific 
therapy  has  been  given  for  a  period  of  six  weeks  and  no 
improvement  has  occurred,  I  have  usually  discontinued  it. 
Occasionally,  however,  success  may  result  in  rare  cases 
from  a  further  continuation  of  the  treatment. 

When  the  general  health  of  the  patient  suffers  as  a  result 
of  the  treatment  it  is,  of  course,  best  to  stop  it  for  a  while 
and  permit  the  patient  to  regain  his  strength  and  then  begin 
it  over  again.  As  a  rule  several  courses  of  treatment  should 
be  given  every  year  for  two  or  three  years.  Gowers  ad- 


PROPHYLAXIS  365 

vises  for  a  period  of  eight  years  after  the  infection  and 
five  years  after  the  last  appearance  of  florid  symptoms  to 
give  iodine  for  several  weeks,  twice  yearly.  Endeavor 
always  to  keep  your  patient  under  observation,  because 
the  tendency  of  syphilis  of  the  nervous  system  to  relapse  is 
great  and  it  lies  in  the  nature  of  the  affection  to  reappear 
in  a  different  form  and  in  another  location. 

Chronic  Intermittent  Treatment. — In  Germany,  A.  Neisser, 
and  in  France,  Fournier,  have  advocated  the  chronic  inter- 
mittent treatment  of  syphilis.  Fournier  sees  in  this  method 
the  only  means  of  limiting  the  development  of  paresis.  It  is 
scarcely  necessary  to  state  that  many  experienced  neurolo- 
gists think  otherwise.  However,  when  such  men  as  Four- 
nier and  Neisser  and  such  experienced  neurologists  as 
Gowers  advocate  this  form  of  treatment,  one  feels  an  obli- 
gation, even  against  his  own  experience  and  judgment,  to 
follow  their  instructions.  Fournier 's  advice  is  in  the  begin- 
ning of  syphilis  to  institute  energetic  mercurial  treatment 
and  continue  it  with  intermissions  for  a  period  of  two  years, 
then  for  a  period  of  two  years  cease  all  antispecific  therapy. 
In  the  fifth  year  after  the  infection  to  give  again  another 
year  of  mercurial  treatment,  which  should  in  the  seventh 
or  eighth  year  after  the  infection  again  be  repeated. 

Objection  has  been  made  to  this  form  of  treatment  be- 
cause of  the  danger  through  its  long  continuance  to  the 
development  of  a  toleration  on  the  part  of  the  specific 
organism  to  the  medication. 

Another  danger  of  too-long-continued  mercurial  and 
iodine  medication  lies  in  the  fact  that  the  tendency  of  the 
individual  to  degenerative  affections  of  the  nervous  system 
may  increase  because  of  a  general  constitutional  weaken- 
ing of  the  system,  since  mercury  and  iodine  are  not  to  be 
regarded  as  indifferent  drugs  to  the  organism.  This  dan- 
ger is  in  reality  not  a  very  great  one. 

A  special  study  of  the  advantages  and  disadvantages  of 
the  chronic  intermittent  method  of  treatment  has  been  made 
by  Perl.  He  comes  to  the  conclusion  that  the  objections 
to  the  method  are  not  well  founded  and  also  that  the  chief 
cause  of  the  appearance  of  tertiary  symptoms  is  due  to 


366  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

insufficient  treatment  during  the  primary  and  secondary 
stages.  It  should  be  mentioned,  however,  that  Perl's  mate- 
rial was  entirely  of  a  tertiary  nature  and  that  his  experi- 
ence does  not  include  metasyphilitic  affections. 

Antimercurialism. — At  the  present  time  the  bitter  fight 
which  was  made  against  the  use  of  mercury  in  syphilitic 
affections  by  such  men  as  Hermann  and  Lorinser  has  been 
almost  forgotten.  The  chief  arguments  of  the  antimercu- 
rialists  were  found  in  the  miners  who  worked  in  the  quick- 
silver mines  and  developed  similar  lesions  in  the  bones  to 
those  occurring  in  syphilis,  and  also  not  infrequently  in 
cases  of  tertiary  and  secondary  lues  the  symptoms  disap- 
peared only  after  a  complete  cessation  of  the  mercurial 
treatment.  The  classical  studies  of  Virchow  and  later 
Kussmaul  have  effectually  contradicted  these  contentions, 
and  they  are  only  interesting  now  historically. 

Refractory  Behavior  of  Genuine  Specific  Processes  to  Mercury 
and  Iodine. — One's  faith  in  the  therapeutic  value  of  mercury 
and  iodine  should  not  be  shaken,  because  occasionally  a 
true  specific  lesion  is  encountered  which  does  not  yield  to 
either  one  of  these  agents.  I  have  had  several  such  experi- 
ences in  which  specific  gummatous  processes,  after  long  and 
energetic  treatment  with  mercury  and  iodine,  remained 
uninfluenced.  One  of  these  lesions  was  located  in  the  optic 
chiasm,  one  in  the  cortex  of  the  frontal  brain,  and  two  were 
found  (postmortem)  on  the  spinal-cord  membranes. 

It  not  infrequently  happens  during  antispecific  treat- 
ment that  new  and  severe  nervous  symptoms  appear. 
Such  observations  are  so  numerous  in  the  literature  that 
only  a  few  can  be  referred  to.  Goldflam  saw  during  the 
course  of  an  energetic  antispecific  treatment  the  develop- 
ment of  a  syphilitic  myelitis,  and  Gowers  reports  a  case  in 
which  a  specific  meningitis  with  gumma  in  the  brain  and 
spinal  cord  appeared  during  antispecific  therapy. 

Optic  Atrophy  a  Centra-indication. — Many  authorities  con- 
sider an  optic  atrophy  as  a  contra-indication  against  the 
use  of  mercury  and  iodine,  because  there  are  numerous  cases 
reported  in  which  the  atrophy  progressed  faster  under 
mercurial  medication.  One  should  remember,  however, 


PROPHYLAXIS  367 

that  an  optic  atrophy  may  be  caused  by  a  primary  degenera- 
tion of  the  optic  fibres,  as  well  as  by  an  interstitial  or 
gnmmatous  optic  neuritis,  and  that  in  the  latter  instance 
energetic  antispecific  therapy  is  especially  indicated. 

Since  one  is  not  always  able,  either  by  the  ophthalmo- 
scopic  picture  or  the  field  of  vision,  to  determine  the  origin 
of  the  atrophic  process,  one  is  often  in  doubt  as  to  the 
proper  course  to  pursue. 

In  such  cases  my  plan  has  been,  after  the  completion  of 
each  cycle  of  inunctions  (four,  five  or  six  inunctions),  to 
take  the  field  of  vision  and  also  make  an  ophthalmoscopic 
examination.  If  this  examination  shows  that  either  the 
ophthalmoscopic  finding  or  the  power  of  vision  is  worse,  I 
discontinue  the  treatment. 

Atoxyl. — Much  has  been  written  during  the  last  few 
years  concerning  the  use  of  atoxyl  in  the  treatment  of  lues. 
The  experiences  of  Bornemann,  Krudener,  Lesser,  Graaf, 
Eobert  Koch,  Fehr,  and  Nonne  show  that  with  large  doses 
and  also  small  ones  an  optic  blindness  may  appear,  which 
frequently  develops  without  premonitory  symptoms  rather 
suddenly,  and  for  this  reason  it  makes  this  agent  danger- 
ous. One  sad  experience  of  my  own  has  caused  me  to  lose 
my  courage  with  regard  to  its  administration. 

Since  the  era  of  salvarsan  this  remedy  has  only  an 
historical  interest. 

Bath  Resorts. — In  cases  where  one  wishes  to  stimulate 
the  eliminative  processes  and  to  increase  the  capacity  of  the 
patient  for  mercurial  treatment  the  famous  sulphur  baths 
of  Aachen,  Tolz,  and  Nenndorf  may  be  recommended.  Afso 
the  thermal  baths  of  Nauheim,  Oeynhausen,  Wildbad- 
Schwarzwald  and  Wildbad-Gastein,  Baden-Baden,  the  baths 
of  Leuk  in  Switzerland  and  at  Wiesbaden,  as  well  as  the 
different  salt-baths,  have  justly  a  good  reputation  for  this 
purpose. 

Zittman  Cure. — The  Zittman  cure  has  been  in  times  past 
often  recommended  in  cases  where  the  symptoms  continued 
refractory  to  mercury  and  iodine,  or  where  these  agents 
were  badly  tolerated.  In  tertiary  symptoms  of  the  mucous 
membrane,  skin,  and  bones  this  method  is  still  made  use  of 


368  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

at  the  present  time,  but  in  specific  disease  of  the  nervous 
system  its  use  is  not  advisable  because  chiefly  of  its  tendency 
to  weaken  the  constitution. 

Tonic  Measures. — It  is  very  important  in  the  treatment  of 
syphilis  of  the  nervous  system  that  the  general  nutrition  and 
strength  of  the  body  should  be  looked  after  by  well-directed 
tonic  measures.  The  neglect  of  this  cardinal  rule  alone 
may  cause  failure  with  the  mercurial  treatment.  The  entire 
hygiene  of  the  organism  must  be  drawn  upon.  The  utiliza- 
tion of  hydrotherapy,  a  strengthening  and  non-irritating 
diet,  abstinence  from  or  moderation  in  the  use  of  alcohol 
and  tobacco,  and  the  avoidance  as  far  as  possible  of  too 
great  mental  activity,  are  to  be  strongly  advised.  Do  not 
forget  to  treat  the  sick  individual  as  well  as  the  sick  body. 
Mental  depression  often  accompanies  a  specific  infection 
and  one  should  always  endeavor  as  far  as  possible  to  combat 
this  in  every  way. 

Psychic  Treatment. — In  the  hypochondriacal  states  which 
are  often  encountered  in  luetics  it  requires  all  the  skill  of 
the  physician  to  successfully  combat  them.  The  patients 
become  syphilophobiacs  and  imagine  themselves  to  be 
tabetic  or  paretic.  They  are  generally  stubborn  cases.  If 
one  does  not  treat  them  specifically  then  they  complain  that 
specific  treatment,  and  that  alone,  is  necessary  for  their 
recovery ;  if  one  treats  them  specifically,  they  also  complain 
that  they  are  still  syphilitic  and  that  their  condition  is  in- 
curable. In  such  cases  it  is  the  physician  and  not  the  medi- 
cation who  must  cure  his  patient,  medicamente,  non-medica- 
mentis.  Not  infrequently  one  observes  that  these  tabes- 
paresis  hypochondriacs  are  finally  correct  in  their  fore- 
bodings and  later  develop  cerebral  and  spinal  disease.  The 
cases  in  which  an  actual  paresis  appears  after  years  of 
hypochondriacal  fears  have  been  observed  by  all  experi- 
enced practitioners. 

Therapeutic  Indications  for  Individual  Forms  of  Nervous 
Lues. — The  treatment  of  arteritic  apoplectiform  attacks 
should  in  the  beginning  be  the  same  as  in  the  non-specific 
apoplexies,  thromboses,  and  emboli.  The  patient  should 


PROPHYLAXIS  369 

have  absolute  mental  and  physical  rest,  cold  to  the  head, 
and  a  non-stimulating  diet.  Only  when  the  apoplectiform 
symptoms  have  passed  over  should  the  antispecific  treat- 
ment be  instituted.  The  specific  treatment  should  first  be 
directed  towards  endeavoring  to  bring  about  a  healing  of 
the  arteritic  process  and  then  later  towards  preventing  an 
extension  of  the  disease  to  other  vessels  and  thus  the  occur- 
rence of  further  apoplectic  attacks. 

Cases  of  relapses  after  energetic  and  long-continued 
treatment  teach  us  unfortunately  that  this  is  not  always 
possible. 

In  the  treatment  of  specific  disease  of  the  peripheral 
nerves  after  the  termination  of  the  antispecific  therapy, 
massage  and  electricity  will  be  found  beneficial. 

In  the  permanent  consequences  of  brain  and  spinal- 
cord  necrosis  one  may  utilize  all  the  therapeutic  aids  which 
are  administered  in  non-specific  hemiplegias  and  para- 
plegias, such  as  hydrotherapy,  massage,  and  electro-  and 
mechano  therapy. 

Sensory  and  motor  symptoms  of  irritation  often  require 
sedative  and  narcotic  treatment.  These  conditions  must  be 
dealt  with  symptomatically. 

Particular  stress  should  be  laid  on  the  care  of  the  blad- 
der function  and  the  avoidance  of  decubitus.  The  bladder 
paresis  often  leads  to  cystitis,  this  extending  upwards  to 
pyelitis  and  pyelonephritis,  and  thus  causes  the  death  of 
the  patient  with  symptoms  of  uraemia  or  sepsis.  On  the 
other  hand,  decubitus,  through  the  weakening  of  the  patient 
or  because  it  brings  about  a  general  sepsis,  produces  of 
itself  the  fatal  termination  in  patients  where  the  spinal 
process  has  been  healed.  I  know  of  many  cases,  both  in 
private  practice  and  in  the  hospital,  where,  by  carefully 
directed  attention  to  the  bladder  function  and  prophylaxis 
against  decubitus,  the  general  health  of  the  patients  was 
maintained  in  fairly  good  condition  for  many  years.  One 
should  endeavor  to  prevent  the  first  beginning  of  a  decu- 
bitus, and  a  commencing  cystitis  may  be  greatly  benefited 
by  washing  out  the  bladder  if  this  procedure  is  carried  out 

24 


370  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

under  the  strictest  aseptic  precautions.  Practical  experi- 
ence has  taught  me  that  failure  in  this  last  injunction  occurs 
more  often  than  one  would  imagine. 

Surgical  Treatment. — In  recent  years  the  surgical  treat- 
ment of  brain  syphilis  has  frequently  been  under  considera- 
tion. It  has  been  previously  stated  that  brain  lues  may 
appear,  presenting  the  symptoms  of  a  brain-tumor,  and 
usually  when  this  occurs  the  symptoms  are  those  of  cortical 
tumors.  There  are  symptoms  of  sensory  and  motor  irrita- 
tion which  may  be  accompanied  with  paralysis,  choked  discs, 
and  occasionally  other  evidence  of  pressure.  Naturally 
when  the  supposed  tumor  has  been  diagnosed  as  specific 
in  nature  antispecific  therapy  will  be  instituted,  but  numer- 
ous experiences  have  taught  us  that  not  infrequently  specific 
tumors  are  refractory  to  treatment ;  they  do  not  yield  to  it. 
Bergmann,  in  the  first  edition  of  his  book  on  brain  surgery, 
advises  against  operation  in  syphilitic  tumors,  but  Horsley 
and  Gowers,  upon  the  basis  of  pathological  studies  which 
proved  gummata  to  be  incurable  by  means  of  mercury  and 
iodine,  recommended  surgical  removal  for  specific  tumors 
which  can  be  reached,  as  the  only  rational  therapy. 

In  1910,  Horsley,  at  the  meeting  of  the  German  Neuro- 
logical Congress  held  in  Berlin,  advocated  trepanning  in 
all  cases  where  gummatous  disease  of  the  brain  could  be 
definitely  determined  and  then  local  treatment  in  the  form 
of  irrigation  with  a  1  to  1000  corrosive  sublimate  solution. 
In  the  discussion  which  followed  strong  antagonism  was 
plainly  manifested  towards  so  radical  a  method  of  treat- 
ment. 

Oppenheim  states  in  his  monograph  on  brain-tumors 
that  Macewen,  Horsley,  Harrison,  Lampiosi,  Parker, 
Eannie,  and  Sands  have  removed  successfully  tumors  of 
syphilitic  origin.  He  further  states  that  Macewen  and 
B  ram  well  advise  that  the  indurations  which  are  often  left 
after  the  termination  of  a  specific  cortical  affection  should 
be  removed  if  they  cause  a  chronic  epilepsy  and  other  severe 
brain  symptoms. 

Oppenheim,  in  1897,  stated  that  there  are  a  number  of 
conditions  in  brain  syphilis  in  which  a  surgical  procedure 


PROPHYLAXIS  371 

may  be  indicated.  Bruns  says  in  his  book  on  tumors  of  the 
nervous  system  that  when  a  specific  affection  assumes  the 
form  of  a  brain-tumor  antispecific  therapy  should  at  first  be 
administered.  If  the  antispecific  treatment  does  not  bring 
about  improvement  inside  of  six  weeks,  then  there  is  time 
to  proceed  with  the  operation. 

In  1898  Schlesinger  and  Friedlander,  after  a  thorough 
study  of  this  question,  came  to  the  following  conclusions : 

Indications  for  surgical  interference  in  known  or  sup- 
posed brain  syphilis : 

1.  Stationary  tumor  after  antispecific  treatment,  which 
is  easily  accessible  and  of  supposedly  small  circumference. 

2.  Progression  of  the  symptoms  in  spite  of  treatment,  if 
an  indicatio  vitalis  exists. 

3.  In  spite  of  antispecific  treatment,   Jacksonian  epi- 
lepsy, although  the  earlier  symptoms  of  tumor  have  disap- 
peared. 

In  a  case  reported  by  Fischer  a  gumma  of  the  dura 
developed  over  the  right  parietal  lobe  broke  down  and  pro- 
duced necrosis  in  the  parietal  bone.  This  abscess  caused  a 
cortical  epilepsy  with  left  hemiplegia  and  unconsciousness. 
Surgical  treatment  of  the  abscess  followed  by  the  adminis- 
tration of  iodine  resulted  in  a  complete  healing  of  the  local 
process  and  a  disappearance  of  the  brain  symptoms.  The 
specific  nature  of  the  tumor  was  confirmed  by  the  appear- 
ance of  fresh  gumnmta  over  both  tibia. 

The  following  case  is  very  instructive  in  this  connection : 
A  man,  forty  years  old,  was  received  into  the  hospital 
with  the  diagnosis  of  idiopathic  epilepsy.  The  history 
showed  that  he  had  suffered  with  epileptic  attacks  for  six 
months,  which  occurred  two  or  three  times  a  week,  and 
bore  the  character  of  severe  idiopathic  epileptic  attacks.  He 
denied  syphilitic  infection.  Still,  a  number  of  consecutive 
abortions  of  his  wife  made  his  history  suspicious.  He  also 
stated  that  during  the  past  few  months  he  had  taken  a 
course  of  inunctions.  The  last  four  weeks  he  had  com- 
plained of  severe  headaches  and  was  apathetic.  On  the  day 
before  his  admission  to  the  hospital  he  had  a  number  of 
severe  epileptic  attacks.  The  objective  examination  was 


372  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

not  entirely  satisfactory  because  of  the  dazed  condition  of 
the  patient  from  the  attacks  of  the  day  before.  It  was 
established,  however,  that  there  were  no  paralyses  of  the 
cranial  nerves  or  of  the  extremities,  and  that  the  cranium 
was  sensitive  to  percussion,  especially  over  the  left  parietal 
bone.  Antispecific  treatment  was  begun,  but  the  same  night 
he  was  seized  again  with  a  succession  of  the  same  epileptic 
attacks,  from  which  he  died. 

The  autopsy  revealed  a  hard  caseous  circumscribed 
tumor  the  size  of  a  walnut,  situated  between  the  dura  and 
the  cortex  over  the  region  of  the  left  paracentral  lobule. 
The  dura  was  thickened  and  fibrous  in  all  directions  extend- 
ing out  from  the  tumor,  and  the  cortex  just  beneath  the 
tumor  was  infiltrated  with  a  caseous  substance.  Careful 
examination  of  all  the  organs  and  tissues  at  the  autopsy 
revealed  no  other  signs  of  either  syphilis  or  tuberculosis. 
The  microscopical  examination  of  the  tumor,  as  well  as  of 
the  infiltrated  cortex,  presented  a  typical  picture  of  a  syph- 
ilitic gummatous  process  with  a  marked  degree  of  endar- 
teritic  and  endophlebitic  changes.  In  this  case  a  timely 
operation  over  the  sensitive  area  of  the  cranium,  followed 
by  an  energetic  mercurial  treatment,  would  in  all  proba- 
bility have  resulted  in  a  complete  recovery. 

Stransky  has  recently  published  an  excellent  monograph 
on  the  subject  of  operative  treatment  in  brain  syphilis.  He 
concludes  that  American  and  English  authorities  take  the 
most  extreme  position  in  favor  of  surgical  interference, 
Bergmann  the  opposite  extreme,  and  Oppenheim,  Bruns, 
and  Henschen  occupy  middle  ground. 

In  a  general  review  of  all  the  pertinent  cases  one  is 
forced  to  the  conclusion  that  there  should  be  no  hesitation 
in  performing  the  operation  when  a  positive  localization 
is  possible  and  antispecific  treatment  does  not  produce  a 
quick  and  definite  response,  or  if  an  indicatio  vitalis  exists. 
Of  course,  after  the  operation  the  antispecific  treatment 
should  be  energetically  continued,  since  it  is  .scarcely  pos- 
sible that  the  extirpated  tumor  is  the  only  manifestation 
of  syphilis  in  the  brain. 


PROPHYLAXIS  373 

A  multiplicity  of  lesions,  as  in  non-specific  tumors,  would 
be  a  centra-indication  against  operative  interference,  as, 
for  instance,  the  complications  of  a  basilar  specific  menin- 
gitis or  an  extensive  specific  lesion  of  the  spinal  cord  with 
the  gummatous  tumor. 

Concerning  surgical  treatment  of  spinal-cord  syphilis, 
so  far  as  my  knowledge  extends,  nothing  is  known. 
We  do  know,  however,  that  spinal  gummata  are  almost 
never  solitary  and  do  not  usually  attain  a  size  sufficient 
to  cause  symptoms  of  compression,  and,  most  important 
of  all,  they  are  probably  without  exception  only  an  accom- 
paniment of  extensive  specific  spinal  changes. 

The  treatment  of  meta syphilitic  affections  of  the  nervous 
system  is  justified  from  the  standpoint  of  the  prophylaxis 
and  especially  so  since  Homen  and  Schuster  have  proven 
that  previous  treatment  of  the  lues  in  tabetic  cases  has 
usually  been  insufficient. 

Dinkier  in  Erb's  clinic  found  many  cases  of  tabes  in 
which  the  marked  improvement  following  mercurial  treat- 
ment did  not  appear  as  merely  coincident.  The  opinions  of 
the  various  authorities  concerning  the  justification  and 
necessity  of  mercurial  and  iodine  treatment  in  true  tabes 
is  as  yet  divided.  For  a  number  of  years  I  have  taken  the 
position  that  every  case  of  tabes  in  which  there  is  a  history 
of  lues,  and  since  the  beginning  of  the  tabetic  symptoms 
has  not  been  thoroughly  treated  with  mercury,  should  re- 
ceive such  treatment  without  further  delay.  If  no  marked 
improvement  results  from  the  treatment,  or  the  disease  does 
not  appear  to  have  been  stopped  in  its  progress,  it  ought  not 
to  be  repeated. 

I  have  had  for  many  years  a  large  number  of  cases  of 
tabes  under  observation  which  apparently  by  the  inunc- 
tion treatment  were  brought  to  a  quiescent  state  and  have 
been  kept  in  this  condition  by  repeating  the  treatment  once 
every  one  or  two  years.  These  are  cases  of  gastric  crisis, 
lightning  pains,  slight  ataxia,  bladder  disturbances,  etc.  I 
have  also  numerous  cases  of  abortive  tabes  under  observa- 
tion which  have  not  developed  but  have  remained  as  rudi- 


374 


SYPHILIS  AND  THE  NERVOUS  SYSTEM 


mentary  cases.  Because  of  these  experiences  I  can  by  no 
means  agree  with  those  who  reject  mercurial  treatment  in 
tabes  dorsalis. 

Erb's  conclusions  on  this  question  are  as  follows: 

1.  In  tabes  with  a  positive  history  of  syphilis  antispe- 
cific  therapy  is  indicated,  with  careful  adaptation,  of  course, 
in  each  case. 

2.  Specially  suitable  are  all  the  recent  cases  of  tabes  in 
which  the  syphilis  does  not  date  too  far  back. 


Syphilis  pure 


Syph  Ills 


Tabes 


Tabes  pure 


FIG.  97. — Minor's  schematic  representation  of  the  justification  and  importance  of  antispecific 
therapy  from  the  time  of  the  syphilitic  infection  to  the  complete  development  of  tabes. 

3.  Further,  all  the  cases  in  which  florid  symptoms  of 
syphilis  can  be  demonstrated  on  other  parts  of  the  body  or 
which  are  complicated  with  symptoms  of  cerebral  or  menin- 
geal  lues. 

4.  Finally,  all  the  cases,  old  cases  as  well,  which  at  the 
time  of  their  syphilis  received  insufficient  treatment. 

E.  Redlich,  as  the  result  of  a  large  clinical  experience, 
in  1910  came  to  the  conclusion  that  an  energetically  re- 
peated mercurial  treatment  in  syphilis  exerted  a  favorable 
influence  towards  preventing  the  development  of  tabes,  but 
that  it  could  not  be  regarded  as  a  certain  prophylactic  meas- 
ure. He  also  believes  that  mercurial  treatment  in  tabes 
itself  is  without  effect,  but  in  early  cases,  however,  it  some- 
times appears  to  induce  a  benign  course  and  perhaps  even 
bring  the  progress  of  the  disease  to  a  condition  of  quies- 
cence. Also  in  acute  relapses  it  seems  at  times  to  have  a 
good  effect.  Mercury  in  such  cases  is  always  to  be  adminis- 
tered in  moderate  doses.  Very  often,  in  fact  too  often,  how- 
ever, it  completely  fails  and  seems  entirely  unable  to  stop 
the  advance  of  the  disease  or  to  prevent  the  appearance  of 
such  severe  symptoms  as  optic  atrophy  or  the  development 
of  a  taboparesis. 


PROPHYLAXIS  375 

Some  authorities  go  still  further  than  Erb  and  recom- 
mend enormous  doses  of  mercury  in  the  form  of  inunctions 
(15  to  20  grammes  pro  die}.  I  can  only  agree  with  Erb  in 
regard  to  the  large  doses  and  consider  their  administration 
a  mistake.  It  may  be  repeated  here  that  general  tonic  and 
constructive  measures,  such  as  hydrotherapy,  electricity, 
massage,  and  constructive  medication,  should  follow  the  in- 
unction treatment. 

In  many  cases  very  good  results  have  been  obtained  by 
me  in  combining  the  mercurial  treatment,  whether  in  the 
form  of  inunctions  or  injections,  with  injections  of  fibro- 
lysin.  These  were  especially  cases  of  marked  cerebral  and 
spinal  paralyses  which  did  not  respond  well  to  mercury  and 
iodine  alone.  In  cases  of  gummatous  meningeal  brain  lues 
after  twelve  injections  of  fibrolysin  I  have  seen  the  paralysis 
disappear.  The  fibrolysin  is  put  up  in  ampules  and  one 
ampule  is  injected  every  four  or  five  days. 

The  treatment  of  congenital  lues  is  practically  the  same 
as  for  the  acquired  form.  Mercury  and  iodine  are  the  spe- 
cifics. Mercury  should  be  given  to  children  by  preference 
in  the  form  of  sublimate  baths  or  in  the  form  of  calomel 
internally.  Iodine  plays  a  more  important  role  in  congeni- 
tal than  in  acquired  syphilis.  In  no  case  of  disease  of  the 
nervous  system  due  to  congenital  syphilis  should  the  ad- 
ministration of  iodine  be  neglected.  The  doses  of  both  mer- 
cury and  iodine  must,  of  course,  be  adapted  to  the  age  of 
the  patient. 

The  postsyphilitic  affections  in  congenital  syphilitics 
should  be  treated  also  in  exactly  the  same  manner  as  the 
same  disease  after  acquired  syphilis.  It  is  particularly 
important  in  children  of  luetic  parents  who  have  manifested 
no  symptoms  of  nervous  syphilis  to  carry  out  a  prophylactic 
nerve  hygiene  whether  the  children  have  or  have  not  shown 
any  evidences  of  neuropathies. 


XIX 

SALVARSAN  THERAPY 

Introduction. — A  special  article  has  been  requested  by  me 
concerning  the  administration  of  salvarsan.  The  recollec- 
tion of  the  great  sensation  that  this  therapeutic  agent  which 
Ehrlich  brought  out  after  painstaking  examinations  and 
experiments  is  still  fresh  in  our  memories.  An  enormous 
clinical  experience  has  been  produced  and  such  a  voluminous 
literature  collected  in  the  last  three  years  that  it  is  almost 
impossible  to  review.  However,  the  work  of  testing  the 
therapeutic  value  of  salvarsan  has  been  pursued  with  such 
vigor  that  at  the  present  time  we  are  able  to  determine 
in  the  main  what  its  chief  advantages  and  disadvantages 
are;  in  other  words,  what  can  be  expected  of  it  and  what 
cannot. 

Dangers. — The  dangers  which  accompany  its  administra- 
tion we  are  able  now  to  say  are  slight,  provided  the  remedy 
is  given  with  a  proper  technique  and  in  the  proper  doses. 

Intramuscular  and  Subcutaneous  Injections. — In  regard  to 
the  technique,  the  large  intramuscular  and  subcutaneous 
injections  which  represented  the  earlier  methods  of  its 
administration  have  been  abandoned.  Experience  has  shown 
that  by  these  methods  of  administration  salvarsan  was  very 
painful,  often  caused  long-continuing  and  sometimes  per- 
manent indurations,  and  not  infrequently  produced  stub- 
born suppurations  and  necroses.  It  was  uncertain  as  to 
dosage  because  one  could  never  determine  how  much  of  the 
injected  remedy  was  going  to  remain  unabsorbed,  deposited 
as  in  a  depot,  in  the  centre  of  the  induration. 

The  numerous  injurious  effects,  in  the  form  of  sciatic 
and  peroneal  paralyses  and  bladder  disturbances,  which 
have  been  ascribed  to  it,  should  be  attributed  to  the  tech- 
nique and  not  the  remedy.  Smaller  doses  are  still  given 
intramuscularly. 

376 


SALVARSAN  THERAPY  377 

Isaac,  in  Lassar's  clinic  in  Berlin,  has  reported  good  re- 
sults with  weekly  intramuscular  injections  containing  0.1 
gramme  of  salvarsan  in  each  injection. 

The  Intravenous  Method. — Salvarsan  at  the  present  time, 
however,  is  administered  far  more  often  intravenously. 
The  advantages  of  this  method  are :  (1)  exactness  in  dosage ; 

(2)  absence  of  pain;  (3)  quicker  distribution  of  the  remedy 
throughout  the  entire  system;  and  (4)  the  certainty  of  ob- 
taining an  effect  from  the  entire  dose. 

There  are  no  dangers  in  this  form  of  administration  if 
(1)  the  remedy  is  injected  in  either  an  alkaline  or  neutral 
solution;  (2)  if  careful  asepsis  or  antisepsis  is  observed; 

(3)  if  care  is  taken  to  prevent  the  entrance  of  air-bubbles 
into  the  veins;  and  (4)  if  it  is  not  given  in  severe  cases  of 
arteriosclerosis,  extensive  nerve  degenerations,  advanced 
cases  of  diabetes,  and  in  general  marked  cachexias,  such  as 
exist  in  tuberculosis  and  carcinoma.    The  danger  which  has 
been  said  to  exist  in  aneurism  and  myocardial  degeneration, 
according  to  my  experience,  has  been  overestimated. 

The  following  illustration  represents  the  apparatus 
which  has  been  used  in  my  clinic  for  the  intravenous  injec- 
tion of  salvarsan.  This  apparatus  was  devised  by  Dr.  A. 
Hauptmann,  assistant  in  the  clinic.  The  apparatus  is  so 
arranged  that  at  any  time  either  the  salvarsan  solution  or 
a  salt  solution  can  be  allowed  to  flow  into  the  vein.  It  is 
extremely  important  that  one  may  be  able  to  determine  at 
first  whether  the  needle  is  really  in  the  vein  or  not  by  a 
trial  test  of  permitting  the  salt  solution  to  flow  first.  In  thin 
veins  especially  one  is  quite  apt  to  puncture  both  walls 
of  the  vein.  Even  a  small  quantity  of  the  salvarsan  solution 
under  the  skin  will  cause  considerable  pain  and  induration 
and  may  lead  to  necrosis.  If  the  salt  solution  flows  into  the 
vein  without  any  subcutaneous  infiltration,  then  the  salvar- 
san may  be  allowed  to  flow,  and  when  it  has  flowed  in  to  the 
amount  of  the  required  dose  which  one  wishes  to  give,  then 
the  salt  solution  should  again  be  turned  on  and  allowed  to 
flow  until  the  salvarsan  has  been  washed  out  of  the  tube  and 
vein  at  the  point  of  injection. 


378 


SYPHILIS  AND  THE  NERVOUS  SYSTEM 


The  apparatus  is  constructed  in  the  following  manner: 
Two  glass  upright  cylinders  are  fused  upon  a  small  hori- 
zontal cylinder,  into  which  is  fitted  a  ground  glass  stop-cock 


a  SALT  SOLUTION 
b   SALVARSAN 
C    CONTROLCYLINDER 
d  STOPCOCK 
e  SALT  SOLUTION  VALVE 
f  SALVARSAN  VALVE 
g  RUBBER  TUBING 
h  GLASS  TUBING 
i    NEEDLE 


FIG.  98. 


which  is  bored  through  in  two  places.  These  openings  in 
the  stop-cock  are  so  arranged  that  when  one  of  them  is 
patent  with  the  cylinder  containing  salvarsan,  salvarsan 


SALVARSAN  THERAPY  379 

flows,  and  when  the  other  opening  is  patent  with  the  cylin- 
der containing  salt  solution,  salt  solution  flows.  Turning 
the  cock  once  around  is  all  that  is  necessary  in  order  to 
change  from  the  salvarsan  to  the  salt  solution.  The  cylin- 
der for  salvarsan  is  graduated  up  to  200  c.c.,  the  one  for  salt 
up  to  100  c.c. 

The  salvarsan  solution  may  be  mixed  in  the  salvarsan 
cylinder  by  shaking  the  powder  in  about  100  c.c.  of  a  hot  salt 
solution  made  from  distilled  water.  This  solution  is  then 
increased  by  the  addition  of  more  salt  solution  of  the  same 
character  and  then  a  15  per  cent,  solution  of  sodium  hydrox- 
ide is  added,  drop  by  drop,  until  a  precipitate  is  formed  and 
the  whole  solution  resembles  an  emulsion.  One  then  con- 
tinues to  add  the  sodium  hydroxide,  drop  by  drop,  shaking 
up  the  solution  well  from  time  to  time,  until  it  becomes  per- 
fectly clear.  The  solution  is  then  either  neutral  or  alkaline 
and  ready  for  administration  without  testing.  If,  however, 
any  particles  are  seen  floating  in  it,  it  should  first  be  filtered 
through  sterile  cotton  before  giving.  At  the  opening  on  the 
bottom  of  the  horizontal  cylinder  a  rubber  tube  is  attached, 
which  contains  on  its  other  end  the  needle,  and  just  behind 
the  needle  there  is  inserted  a  small  glass  tube.  The  glass 
tube  in  this  position  enables  one  to  see  that  all  the  air  is 
out  of  the  apparatus  and  also  helps  one  to  ascertain  when 
the  vein  is  punctured  rightly  by  the  backward  flow  of  blood 
into  the  tube.  At  the  first  appearance  of  blood  in  this  tube 
the  ligature  around  the  arm  should  be  released  instantly. 
One  of  the  large  veins  at  the  elbow  should  be  selected  by 
preference  for  the  injection  of  the  salvarsan  solution.  If  the 
veins  in  this  locality  are  not  suitable  a  vein  may  be  utilized 
in  any  other  part  of  the  arm  or  leg  where  one  is  found  that 
is  accessible.  The  ligature  should  be  placed  around  the 
arm  just  above  the  elbow,  rather  tight,  in  order  to  congest 
the  veins,  but  not  tight  enough  to  shut  off  the  pulse  at  the 
wrist.  This  ligature  should  be  tied  in  such  a  manner  that 
it  can  be  quickly  released.  Directing  the  patient  to  make  a 
fist  causes  the  veins  to  stand  out  still  more  prominently 
and  thus  makes  them  easier  of  access  for  puncture.  The 
needle  should  be  directed  as  nearly  horizontal  with  the  vein 


380  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

as  possible  in  order  to  avoid  puncturing  the  opposite  side  of 
the  vein-wall.  Oftentimes,  either  on  account  of  the  thinness 
of  the  veins,  or  a  venosclerosis  where  the  vein  is  so  resistant 
that  it  continually  rolls  out  from  under  the  needle,  or  be- 
cause the  vein  is  so  covered  by  fat  that  it  cannot  easily  be 
located,  it  is  best  to  cut  down  on  the  vein  and  plainly  expose 
it.  This  can  be  done  by  using  a  1  per  cent,  solution  of 
novocaine  for  local  anaesthesia.  It  is  much  better  to  do  this 
than  to  continue  making  futile  attempts  to  enter  veins  with 
resistant  walls  or  those  lying  deep  in  the  tissues  and  difficult 
to  palpate. 

Complications  Following  the  Administration  of  Salvarsan. — 
After  the  administration  of  a  dose  of  salvarsan  the  patient 
should  remain  in  bed  24  hours ;  then  if  no  fever  develops  he 
may  get  up  and  resume  his  customary  occupation.  Some- 
times considerable  fever,  the  temperature  rising  as  high  as 
103°  or  104°  Fahrenheit,  follows  the  adminstration  of  sal- 
varsan intravenously.  Such  cases  are  exceedingly  rare, 
however,  and  may  be  almost  entirely  avoided  if  freshly  dis- 
tilled water  is  used  in  the  making  up  of  the  physiological 
salt  solution.  The  water  impurities,  to  which  alone  Wech- 
selmann  wished  to  attribute  the  complications  following  the 
administration  of  salvarsan,  has  been  shown  by  Finger,  and 
also  by  Westphal  from  recent  experimental  examinations, 
not  to  possess  the  importance  which  Wechselmann  desired 
to  give  to  them.  Now  and  then  cases  of  diarrhoea,  vomiting, 
and  a  scarlatina-like  rash  still  occur,  which,  however,  be- 
cause of  their  comparative  infrequency  and  freedom  from 
danger,  should  not  be  regarded  as  of  much  importance. 

Is  Salvarsan  Injurious  to  the  Nervous  System? — The  ques- 
tion as  to  whether  salvarsan  in  itself  is  injurious  to  the 
nervous  system  deserves  special  attention.  While  Ehrlich 
in  his  first  publications  had  cautioned  against  the  use  of 
salvarsan  in  well-advanced  processes  of  nervous  degenera- 
tion, he  maintained  that  the  remedy  for  a  healthy  nervous 
system  was  harmless.  In  the  very  beginning,  the  medical 
profession,  because  the  remedy  was  an  arsenic  preparation 
and  because  of  their  recent  experiences  with  atoxyl,  were 


SALVARSAN  THERAPY  381 

suspicious  of  its  influence  on  the  optic  nerve.  Robert  Koch, 
in  his  extensive  experience  with  atoxyl  in  treating  the  sleep- 
ing-sickness, demonstrated  its  influence  on  the  optic  nerve 
to  be  unusually  harmful.  I  made  the  first  pathological  ex- 
amination in  two  optic  nerves,  as  well  as  the  primary  centres 
of  vision  belonging  to  these  nerves.  The  case  was  one  of 
inoperable  metastatic  carcinoma  of  the  uterus,  in  which 
atoxyl  had  been  administered  subcutaneously  daily  in  ordi- 
nary doses.  It  has  been  determined  that  salvarsan  is  not 
injurious  when  the  optic  nerve  is  intact,  and  also  that  non- 
specific disease  of  the  optic  nerve  appears  to  progress  no 
faster  after  the  administration  of  salvarsan  than  before. 

On  the  other  hand,  since  the  beginning  of  the  salvarsan 
era,  disease  of  the  acoustic  and  facial  nerves  appears  more 
often  than  before.  Finger,  of  Vienna,  was  the  first  to  call 
our  attention  to  this  and  attributed  the  more  frequently 
occurring  paralysis  of  these  cranial  nerves  directly  to  the 
toxic  effect  of  salvarsan. 

At  the  annual  meeting  of  the  German  neurologists,  in 
October,  1911,  at  Frankfort-a.-M.,  an  extensive  discussion 
took  place  between  the  advocates  and  the  opponents  of 
Ehrlich's  remedy.  Shortly  before  this  meeting  fatal  cases 
as  a  result  of  encephalitis,  cases  of  acutely  developing 
spinal  paraplegia,  and  many  cases  of  peripheral  neuritic 
paralysis,  had  been  reported.  It  was  also  shown  at  that 
meeting  that  cases  of  primary  and  secondary  syphilis  could 
not  be  primo  ictu  cured  by  salvarsan,  that  a  "therapia 
sterilisans  magna"  in  the  sense  of  Ehrlich  could  not  be 
accomplished.  At  that  time  cases  of  the  so-called  (neuro- 
recidive)  relapses  in  nervous  tissue  had  already  been  pub- 
lished by  Geronne,  Gutmann,  Dessneuse,  Dujardin, 
Assmann  and  Nonne,  in  which  the  examination  of  the  spinal 
fluid  by  the  presence  of  lymphocytosis,  Phase  I,  and  the 
Wassermann  reaction  in  the  spinal  fluid  had  proven  that 
these  were  cases  of  true  brain  syphilis.  The  fact  that  the 
(neuro-recidive)  nervous  recurrences  in  general  disappear 
under  salvarsan  and  mercurial  therapy  is  another  proof 
that  they  are  cases  of  brain  lues.  Since  then  Klineberger, 


382  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

Dreyfus,  Yaloziceky,  and  others  have  confirmed  this  opin- 
ion. At  the  present  time  the  consensus  of  opinion  in  regard 
to  the  nervous  recurrences  may  be  expressed  as  follows: 

More  often  than  formerly  since  the  introduction  of  sal- 
varsan  in  the  therapy  of  syphilis  of  the  nervous  system  one 
sees  various  paralyses  of  the  cranial  nerves.  A  portion  of 
these  paralyses  is  only  apparently  of  more  frequent  occur- 
rence, because  since  the  beginning  of  this  salvarsan  contro- 
versy the  acoustic  nerve,  and  particularly  its  labyrinthine 
branches,  have  been  much  more  carefully  observed  than 
heretofore.  One  must  admit  that  salvarsan  is  able  to  ren- 
der spirochaete  foci  mobile  or  active  which  otherwise 
perhaps  might  have  remained  latent.  According  to  Ehr- 
lich's  opinion  these  latent  foci  are  found  particularly  in 
those  places  where  the  salvarsan  solution  is  impeded  in  its 
circulation  and  as  a  result  of  this  the  remedy  is  able  to 
attack  the  spirochaete  only  to  a  limited  extent.  For  example, 
the  various  bony  canals  and  foramina  through  which  the 
cranial  nerves  course  offer  suitable  conditions  for  prevent- 
ing thorough  contact  of  the  spirochaete  foci  with  the 
salvarsan. 

The  adherents  of  Ehrlich  say  that  this  quality  of  salvar- 
san to  arouse  a  latent  cerebral  lues  out  of  its  latency  and 
cause  it  to  become  active  is  an  advantage,  because  one  in 
this  way  learns  of  the  position  of  the  enemy.  On  the  other 
hand,  the  opponents  assert  that  an  organically  specific  dis- 
eased organ,  and  especially  an  organically  specific  diseased 
nervous  system,  possesses  the  tendency  to  relapses,  and 
that  therein  lies  the  danger  of  salvarsan,  that  a  state  of 
permanent  quiescence  has  not  been  produced. 

The  Influence  of  Salvarsan  on  Syphilogenetic  Diseases. — The 
innumerable  reports  concerning  the  effect  of  salvarsan  in 
the  last  four  years  permit  us  to  say  that  the  specific  syph- 
ilitic affections  frequently  are  very  favorably  and  speedily 
influenced,  but  that  tabes  and  paresis  show  no  material 
change  in  their  clinical  course.  There  are  authorities  who 
in  incipient  cases  of  tabes  and  paresis  have  observed  a  con- 
dition of  quiescence  or  stand-still  and  very  marked  remis- 
sions in  both  of  these  affections,  but,  on  the  other  hand, 


SALVARSAN  THERAPY  383 

other  observers  have  reported  only  unfavorable  results, 
more  marked  progression  and  the  appearance  of  new 
symptoms  directly  following  the  administration  of  the 
salvarsan. 

Concerning  the  influence  of  this  remedy  on  the  four 
reactions,  the  following  can  be  said : 

In  true  cerebrospinal  syphilis,  if  the  case  recovers  clini- 
cally the  four  reactions  gradually  become  lessened  in  inten- 
sity and  may  entirely  disappear.  These  cases  are  the  ideal 
cases,  of  which  I  have  seen  a  large  number. 

In  tabes  and  paresis  the  influence  of  salvarsan  on  the 
four  reactions  is  slight  or  none  at  all,  and  this  is  true  not 
only  for  the  cases  which  are  progressive  and  show  no  im- 
provement, but  also  for  those  which  manifest  clinically  im- 
provement and  even  quiescence.  Even  here  parasyphilis 
behaves  differently  from  specific  syphilis.  Taking  every- 
thing into  consideration,  it  must  be  said  that  advantages 
which  are  free  from  objection  cannot  be  ascribed  to  salvar- 
san that  mercury  does  not  also  possess.  One  must  admit 
that  where  quick  effect  is  necessary  salvarsan  is  to  be  pre- 
ferred, but,  on  the  other  hand,  it  should  be  strongly  empha- 
sized that  where  vital  centres  are  affected,  such  as  the 
medulla  and  the  upper  cervical  cord,  salvarsan  is  contra- 
indicated.  This  view  must  be  taken  since  A.  Westphal  and 
Stertz  reported  their  case  of  meningomyelitis  of  the  cervical 
cord,  which  under  salvarsan  therapy  was  transformed  into 
an  acute  condition  that  may  be  regarded  as  the  Herx- 
heimer  reaction,  and  caused  the  death  of  the  patient. 

It  should  be  acknowledged  that  salvarsan  in  many  cases 
exercises  a  general  tonic  action. 

Recapitulation. — Summarizing  it  can  be  said  at  present 
that  in  cerebrospinal  syphilis  in  individual  cases  a  quicker 
and  more  far-reaching  result  may  be  attained  with  salvar- 
san than  with  mercury  and  iodid.  There  are  also  cases  in 
which  salvarsan  is  effective  after  mercury  and  iodid  have 
failed  or  had  an  insufficient  effect.  The  cases  in  which  sal- 
varsan has  had  an  entirely  favorable  effect,  however,  are 
in  the  minority.  In  by  far  the  greater  number  of  cases  the 
superiority  of  salvarsan  over  mercury  and  iodid  is  not 


384  SYPHILIS  AND  THE  NERVOUS  SYSTEM 

apparent.  In  cases  of  gummatous  disease  of  the  central 
nervous  system  immediate  salvarsan  treatment  may  be  said 
to  be  specially  indicated.  In  specific  disease  where  the  vital 
centres  are  involved  it  is  especially  contra-indicated. 

Tabes  Dorsalis. — Salvarsan  may  in  general  be  given,  in 
tabes,  in  those  cases  where  mercury  is  indicated,  also  in 
cases  where  no  antispecific  therapy  has  been  administered 
since  the  beginning  of  the  spinal-cord  affection.  Whether 
salvarsan  is  to  be  preferred  where  the  disease  is  rapidly 
progressive  in  character,  or  symptoms  of  irritation  are  a 
prominent  feature,  has  not  as  yet  been  determined.  It  is 
probably  indicated  in  those  cases  in  which  a  certain  degree 
of  cachexia  sets  in  early.  It  is  also  to  be  recommended  as 
well  as  mercury  in  cases  that,  under  the  chronic  intermittent 
treatment,  have  run  a  benign  course. 

Optic  atrophy  is  not  a  contra-indication,  providing  small 
doses  are  given.  Centra-indications  are  found  when  the 
disease  affects-  the  upper  cervical  region  and  the  medulla 
oblongata  where  the  vital  centres  are  situated. 

That  salvarsan  is  of  any  more  value  in  tabes  than  mer- 
cury has  not  as  yet  been  proven. 

Paresis. — The  treatment  of  incipient  cases  of  paresis 
with  salvarsan  is  permissible.  In  those  cases  where  mer- 
cury has  had  a  favorable  influence,  but  where  it  seems  no 
longer  to  benefit,  salvarsan  is  to  be  recommended.  In  ad- 
vanced cases  where  mercury  has  been  tried  without  effect 
the  administration  of  salvarsan  is  useless.  In  advanced 
cases,  contrary  to  Ehrlich's  first  warning,  salvarsan  may  be 
given  without  injury. 

Up  to  the  present  time  no  case  of  so-called  (neuro- 
recidive)  nervous  relapse  has  been  reported  in  a  case  of 
paresis.  Cases  have  been  reported,  however,  in  which, 
shortly  after  the  treatment  with  Ehrlich's  remedy,  severe 
maniacal  symptoms  have  suddenly  appeared  which  made 
necessary  the  confinement  in  an  institution  of  the  previously 
free  patient.  This  experience  has  occurred  twice  in  my 
practice.  This  excited  state,  however,  as  is  well  known, 
is  apt  to  occur  in  any  case  of  paresis,  either  with  or  without 
treatment.  It  must  be  said  that  the  most  observations  in 


SALVARSAN  THERAPY  385 

this  affection  are  of  a  too  recent  date  to  draw  definite  con- 
clusions in  regard  to  them.  For  instance,  one  observer 
writes:  "In  a  lady  in  whom  paresis  was  diagnosed  within  a 
period  of  ten  days  a  complete  recovery  took  place. ' ' 

The  consensus  of  opinion  at  the  present  time  is  that 
salvarsan  is  no  more  of  a  cure  for  paresis  than  mercury  and 
iodid.  Cases  have  been  observed  in  which  marked  im- 
provement has  followed  soon  after  the  treatment,  in  which 
the  speech  and  hand-writing,  and  in  fact  the  entire  mental 
state,  appeared  to  undergo  a  decided  change  for  the  better. 
There  are  no  cases  reported,  however,  in  which  the  improve- 
ment has  been  any  more  marked  than  many  experienced 
practitioners  have  seen  before  the  salvarsan  era. 

Dosage. — In  the  beginning  of  our  experience  with  sal- 
varsan large  doses,  0.6,  0.8,  1  gramme,  were  given  and 
repeated  three  or  four  times.  Later  smaller  doses,  0.2,  0.3, 
0.4  gramme,  were  administered  once  every  seven  or  eight 
days  and  repeated  four  or  five  times.  For  the  last  two  years 
the  combined  treatment  of  salvarsan  and  mercury  has  found 
the  most  favor.  One  combines  at  present  the  salvarsan 
injection  with  mercurial  inunctions  and  calomel  injections. 
Recently  Dreyfuss,  in  Frankfort-a.-M.,  has  recommended 
again  large  doses  of  salvarsan.  He  administers  in  a  period 
of  from  five  to  eight  weeks  as  much  as  5  grammes  of  sal- 
varsan. In  the  intervals  between  the  salvarsan  infusions 
he  injects  large  doses  of  calomel.  Since  Dreyfuss  has  ob- 
served no  injurious  results  from  this  treatment,  and  since 
he  has  worked  using  as  control  the  lumbar  puncture,  and 
the  reactions  in  the  spinal  fluid  have  been  favorably  in- 
fluenced, this  method  of  treatment  is  worthy  of  trial.  At  the 
present  time  I  am  using  this  treatment  in  my  department 
at  Eppendorf,  but  thus  far  have  formed  no  conclusions. 
I  can  say  this  much,  however,  that  up  to  the  present  time 
I  have  not  seen  any  progression  of  the  symptoms  in  any 
patient  who  has  been  under  this  treatment. 

Salvarsan  in  Congenital  Syphilis. — As  mercury  and  iodid 
is  administered  in  the  same  way  in  congenital  syphilis  as 
in  the  acquired  type,  so  also  the  indications  for  salvarsan 
in  both  these  forms  of  syphilis  are  the  same. 


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INDEX 


Abscess    brain,    differentiation    from 

specific  cerebral  meningitis,  93 
Alcoholism,  chronic,  differentiation  be- 
tween congenital  lues  and 
the  effect  of  alcohol  on  off- 
spring, 328 
from  paresis,  203 
influence  of,  on  nervous  system, 

59 

Amaurptic  idiocy,  334 
Amentia  in  syphilis,  179 
Amyotrophic  lateral  sclerosis  and  syph- 
ilis, 248 

Aneurism  in  syphilis,  41 
Aortitis,  differentiation  of  specific  from 

non-specific  type,  40 
spirochsete  pallida  in,  40 
Wassermann  in,  41 

Apoplexy,  cerebral,  in  congenital  syph- 
ilis, 317 
differential  diagnosis  from  specific 

basilar  meningitis,  141 
Arteries    and    blood-vessels,     specific 

disease  of,  33 

disease  of,  on  the  brain  base,  65 
rupture  and  secondary  hemor- 
rhage in,  43 
Arteriosclerosis  in  syphilitics,  38 

differential  diagnosis  from  specific 
meningitis  of  the  convexity, 
74 

from  paresis,  198 
Arteritis  in  cerebral  syphilis,  60 
apoplectic  symptoms  in,  67 
age  of  patients  in,  61 
blood-pressure  in,  66 
diagnosis  of,  72 
differential  diagnosis  of,  72 
dizziness  in,  62 
headache  in,  62 
hemianopsia  in,  69 
insomnia  in,  62 
motor  irritation  in,  66 
paralysis  in,  66 
pons  involvement  in,  68 
prodromal  symptoms  in,  61 
prognosis  in,  160 
psychic  symptoms  in,  62 
summary  in,  75 
Atrophy,  optic  spinal  form,  103 

Blood,  changes  in,  syphilis,  168 
channels,  diseases  of,  169 
pressure  variation  in  specific  arte- 
ritis,  66 


Brown-Sequard  symptom  complex  in 
syphilis,  249,  277 

Carcinoma,  differential  diagnosis  from 

cerebrospinal  syphilis,  296 
Catatonia  in  syphilis,  178 
Cerebrospinal  meningitis,  specific  (see 

Meningitis) 
Chiasm,    optic,    specific    disease    of, 

110 

Chorea  in  syphilis,  172 
Choked  disc,  explanation  of,  100 

in  meningitis  of  the  convexity, 

84 

influence  of  treatment  on,  105 
ophthalmoscopic  changes  in,  99 
Convulsions,  cortical,  84 
Cornil,  teaching  of,  35 
Cranium,  pathology  of,  15 
Cytodiagnosis,     see     Lymphocytosis, 
341 

Deafness  in  acquired  lues,  145 

in  congenital  lues,  326 
Dementia  paralytica,  182 

antispecific  treatment  in,  190 
differential  diagnosis  in,  191 
early  development  of,  182 
increase,  182 
infantile    and     juvenile     fprm, 

185 

neuropathic  inheritance  in,  187 
other  causes  of,  183 
paresis  and  specific  brain  dis- 
ease, 186 
pathology  in,  188 
recovery  in,  193 
relationship    to    syphilis,    182, 

190 

salvarsan  in,  384 
Wassermann  in,  349 
Dementia  in  syphilis,  179 
Diabetes  insipidus  and  mellitus  in  spe- 
cific basilar  meningitis,  151,  153 
Dyscrasia  as  result  of  antispecific  ther- 
apy, 169 

Edinger's  exhaustion  theory,  54 
Ehrmann's  theory,  54 
Encephalitis,  simple,  48 . 
after  salvarsan,  381' 
Encephalomalacia  in  syphilitics,  201 
Endarteritis,  see  Arteries  and  Blood- 
vessels, 33 
Endophlebiti§,  see  Veins,  37 

401 


402 


INDEX 


Epilepsy  in  syphilis,  172 

in  congenital  syphilis,  323 
Jacksonian,  85 
in  tumor  cerebri.  92 
Epileptic  convulsions,  general,  of  corti- 
cal origin,  90 

Etiology,  factors  causing  nervous  syph- 
ilis, 12 
Extragenital  infection,  8 

Familiar  appearance  of  syphilogenetic 
diseases,  53 

Fibrolysin  in  combination  with  mer- 
cury in  the  treatment  of  brain-syph- 
ilis, 375 

Friedreich's  ataxia  in  congenital  syph- 
ilis, 335 

Fuchs-Rosenthal  method  of  counting 
lymphocytes,  341 

Gastric  crises,  isolated,  274 
Gliosis,  central,  with  disease  of  pos- 
terior columns,  249 
Globulin  test,  Phase  I,  341 

significance  of,  346 
Glycosuria  in  congenital  lues,  325 

in     specific     basilar     meningitis, 

151 
Gray   substance   of   cord,   disease  of, 

246 
Gummata,  description  of,  24 

isolated  gummata,  prognosis  in,  159 
location  of,  20 
regressive  changes  in,  26 
secondary  changes  in  brain  sub- 
stance from,  26 
size  of,  19 
in  spinal  cord,  215 
surgical  treatment  of,  370 
Gummatous  processes,  4 

Hsemolysin,  significance  of,  in  spinal 

fluid,  354 

Head  injury,  influence  of,  on  the  causa- 
tion of  brain  syphilis,  59 
Headache  in  specific  arteritis,  62 

in  specific  basilar  meningitis,  96 
in  specific  meningitis  of  the  con- 
vexity, 78 

in  pachymeningitis  alcoholica,  92 
Hemianopsia,  110 

different  forms  of,  111 
oscillating,  112 
Hemiepilepsy,  85 

Hemiopic  reaction  of  the  pupils,  113 
Hemiplegia  in  specific  arteritis,  67 
Heubner's  arteritis,  4 

teaching  of  Heubner,  34 
Hydrocephalus     in     congenital     lues, 

315 

Hypochondria  in  syphilis,  174 
Hysteria  in  syphilis,  171 

in  congenital  syphilis,  321 
in  spinal  syphilis,  282 


Idiocy  in  hereditary  lues,  322 

with  spastic  paralysis,  332 
Infection,  specific,  extragenital,  8 
ignorance  of,  8 
influence  of,  on  later  developing 

brain  syphilis,  157 
proof  of,  7 

Insomnia  in  specific  arteritis,  62 
Intercostal  neuralgia  in  syphilis,  302 
Iodine  in  syphilis,  362 
Ischuria  paradoxia  in  specific  lumbar 
myelitis,  227 

Korsakoff's  symptom-complex  in  brain 

syphilis,  180 
Koster,  teaching  of,  in  specific  arterial 

disease,  36 

Labyrinth,  specific  disease  of,  146 
Landry's  paralysis  and  syphilis,  244 
Lumbar  puncture,  339 

admixture  of  blood  in,  340 
dangers  in,  339 
spinal  pressure,  339 
typical  findings  in  spinal  fluid 
in  cerebrospinal  syphilis,  pare- 
sis and  tabes,  352,  353 
value  of   four  reactions  in  dif- 
ferential diagnosis,  351 
Lymphocytes,  origin  of,  346 

type  of  cells,  346 
Lymphocytosis,  significance  of,  345 

Mania  in  syphilis,  175 
Manic-depressive  insanity,  176 
Medulla,  specific  disease  of,  150 
Melancholia  in  syphilis,  175 
M£niere's  symptom-complex  in  syph- 
ilis, 147 

Meningitis,  basilar  syphilitic,  96 
clinical  symptoms  in,  96 
differential   diagnosis    from 

brain  tumor,  138 
cysticercus,  140 
multiple  sclerosis,  140 
relapsing  oculomotor  pa- 
ralysis, 141 
tubercular  and  epidemic, 

141 

eye  muscle  paralysis  in,  116 
hemianppsia  in,  110 
hemippic  reaction  in,  113 
location  of,  30 
neuritis  descendens  in,  110 
ophthalmoscppic  and  perimet- 

rical  examination  in,  103 
optic   atrophy   and   neuritis, 

110 

pons    and    medulla    involve- 
ment, 149 
prognosis,  153 
psychic  disturbances  in,  96 
visual  disturbances,  110 


INDEX 


Meningitis    of    the   brain    convexity, 

symptoms  in,  78 
differential  diagnosis  in,  93 
cerebral,  syphilitic,  symptomatol- 
ogy, 76 
cerebrospinal,  285 

differential  diagnosis,  296 
pathology  in,  286 
postsyphilitic  changes  in,  286 
summary  of  symptoms,  294 
gummatous    and    fibrous    hyper- 
plastic  form,  28 
injury  to  the  structures   on 

the  base  from,  31 
the  leptomeninges  in,  28 
localization  of,  30 
simplex  in  luetics,  30 
spinalis,  see  Spinal  Cord,  221 
Metasyphilitic    disease    in    congenital 

lues,  329 

antispecific  treatment  in,  373 
Migraine  and  syphilis,  204 

in  congenital  syphilis,  322 
Multiple  sclerosis,  see  Sclerosis 
Myelitis,  acute  transverse,  236 
course  of,  237 

differential  diagnosis  in,  240 
pathology,  241 
chronica  syphilitica,  215 

see  also  Spinal  Cord,  221 
transverse,  216 
meningo-,  224 
cervical,  227 
course  of,  237 
dorsal,  225 
lumbar,  227 
symptoms  in,  224 
simple,  243 

differential  diagnosis  from  com- 
bined tract  disease,  255 
multiple  sclerosis,  252 
spinal  tumor,  255 
Myosis,  130 

Nerve  nuclei,  primary  degeneration  of, 

48 
Nerves,  abducens,  course  of,  119 

disease  of,  135 
auditory,  disease  of,  145 
cranial  involvement  of,  in  basilar 

meningitis,  97 
in  congenital  lues,  324 
olfactory  disease  of,  97 
facial,  disease  of,  143 
hypoglossal,  vagus  and  glossopha- 

ryngeal,  disease  of,  147,  148 
neuralgia  of,  301 
brachial  plexus,  302 
the  cervical,  302 
intercostal,  302 
lumbar  and  sacral,  302 
oculomotor,  blood  supply  of,  118 
complete  paralysis  in,  128 
course  of,  116 


Nerves,  oculomotor,  disease  of,  128 

of  nucleus,  129 

frequency  of  paralysis  in,  127 
involvement  of  internal  branches, 

129 

isolated  paralysis  in,  129 
nuclear  and  peripheral  paralysis 

and  perineuritis  in,  128 
secondary  disease  of,  128 
subdivisions  of  nucleus,  117 
summary,  135 

optic,  involvement  in  basilar  men- 
ingitis, 97 

in  brain  syphilis,  108 
in  congenital  syphilis,  325 
mercurial  treatment  in  atro- 
phy of,  366 
neuritis  in,  100,  110 
trigeminal,  disease  of,  142 

neuralgia  in,  301 
trochlear,  course  of,  119 

paralysis  of,  136 
Nervous  disease,  increase  of,  2 

organic,    nonspecific   in    origin, 

11 
Nervous  system,  primary  disease  in, 

47 
Neurasthenia,  cerebral,  in  syphilis,  171 

differential  from  paresis,  202 
Neuritis,  mercurial,  361 
multiple,  301,  303 
multiple  root,  307 
specific  peripheral,  300 
of  spinal  roots,  216,  299 
Neuropathic  heredity,  influence  of,  in 

nervous  lues,  60 
on  paresis,  187 
Neurosis  in  syphilis,  166 

in  congenital  syphilis,  320 
Nystagmus,  136 

Ophthalmoplegia  externa,  129 

interim,  129,  133 

in  migraine,  206 

progressive,  138 
Osteochondritis  in  congenital  syphilis, 

326 

Osteomyelitis  in  syphilis,  15 
Ostitis,  15 

Pachymeningitis  alcoholica,   headache 

in,  92 
with    paralysis    of    the    glosso- 

pharyngeal,  148 
in  congenital  syphilis,  317 
Paralysis,  of  eye  muscles  in  nervous 

syphilis,  116 

description  of,  in  brain  syphi- 
lis, 126 

differential  diagnosis  in,  137 
late  appearance  of,  127 
oculomotor,  127 
significance  of,  119 


404 


INDEX 


Paralysis,    progressive,    see   Dementia 

Paralytic^,  182 
specific  spinal,  Erb's,  232 
Paranoia  in  syphilis,  178 
Patellar   reflexes    in    meningomyelitis, 

228 

in  tabes,  273 

Pathology  of  cerebrospinal  syphilis,  286 
combination  of  differential  forms 

of  nervous  lues,  44 
of  congenital  syphilis,  314 
differential    diagnosis  of  syphilis 

by,  from  sarcoma,  44 
cysticercus,  44 
tuberculosis,  45 
of  nervous  syphilis,  15 
of  spinal  syphilis,  219,  241 
Peduncles,  cerebral  disease  of,  149 
Perineuritis    of    orbital    branches    of 

oculomotor,  128 
Periphlebitis,  37 
Pia  mater,  cerebral  inflammation  of, 

28 

spinal,  213 

Poliomyelitis  and  syphilis,  245 
Polydipsia  and  polyuria  in  specific  basi- 

lar  meningitis,  97 
Polyncuritis,  301,  303 
see  also  Neuritis 
Pons,  disease  of,  149 

blood  supply  in,  149 
in  specific  arteritis,  68 
Postmortem  findings  with  a  negative 

history  in  syphilis,  9 
Pseudobrain  tumors,  106 
Pseudoparesis,  196 
Pseudoparalysis  in  congenital  syphilis, 

326 

Pseudosystem  disease,  255 
Pseudotabes  syphilitica,  275 
in  congenital  lues,  331 
Psychic     disturbances    in     congenital 

syphilis,  322 
of  the  convexity,  79 
in  meningitis  of  the  brain  base, 

96 

in  syphilis  in  general,  168 
Ptosis  in  brain  syphilis,  129 
Pupil  anomalies,  in  alcoholics,  131 
in  congenital  syphilis,  324 
in  meningitis  of  the  convexity, 

81 

in. normal  persons,  130 
in  syphilitics,  130 

Rachialgia,  309 
Rachitis,  nervous  form  of,  319 
Ranvier,  teaching  of,  in  arteritis,  35 
Reactions,  the  four  in  differential  diag- 
nosis, 351 

in  prognosis,  353 

typical  findings  of,  352 
Regio  interpeduncularis,  65 
Residuals,  after  syphilis,  10 


Salvarsan,  see  Therapy,  376 
Sarcoma  of  the  spinal  cord  resembling 
the  clinical  picture   of  syphilis,   5, 
296 
Sarcomatous  infiltration  of  meninges  of 

brain  and  cord,  diffuse,  44 
Sclerosis,  disseminated,  in  congenital 

syphilis,  335 

multiple,  differential  diagnosis  from 
specific  basilar  meningitis, 
140 

from  cerebrospinal  syphilis,  295 
from  specific  myelitis,  280 
in  congenital  lues,  335 
symptoms  of,  252 
Sensory  disturbances,  cortical,  90 
Serum  diagnosis,  see  Wassermann  Reac- 
tion, 344 
Speech  disturbances  in  specific  arteritis, 

68 
in  meningitis  of  the  convexity, 

90 
Spinal  cord,  syphilis  of,  207 

disease  of  the  blood-vessels  in, 

214 

differential  diagnosis  in,  278 
of  the  gray  substance,  215 
of  the  membranes,  211 
myelitis,  215 
pathology  in,  219 
prognosis,  282 
spinal  meningitis,  221 
symptomatology,  220 
fluid,  see  Lumbar  Puncture,  328 
paralysis,  Erb's  syphilitic,  232 
pressure,  339 

root,  symptoms  caused  by,  257 
Spirochsete  pallida  in  aortitis,  40 
in  congenital  lues,  310 
discovery  of,  7 
in  gummatous  nodules,  26 
in  the  nerve  bundle,  55 
Statistics  of  syphilis,  2 
in  paresis,  183 
of  the  prognosis  of  nervous  lues, 

154,  161-165 
of  syphilitic  disease  of  the  nervous 

system,  3 
in  tabes,  260 
Syphilis,    cerebral    and    cerebrospinal, 

see  Meningitis,  76,  285 
of  the  cranium  and  vertebrae,  15-16 
extragenital  infection,  8 
history  of,  4 
increase  of,  1 
the  infectious  factor,  5 
ignorance  of  infection,  8 
law  of  the  gradual  attenua- 
tion of  syphilitic  virus,  311 
past  evidences  of,  10 
proof   of,    syphilitic    findings 
post  mortem  with  negative 
history,  9 
transmission  of,  to  monkeys,  6 


INDEX 


405 


Syphilis,  hereditary,  310 

defective  development  in,  315 
differential  diagnosis,  328 
eye  symptoms  in,  314 
Friedreich's  ataxia  in,  335 
Homen's  familiar  disease  in,  332 
hydrocephalus  in,  315 
involvement  of  internal  organs 

in,  312 

late  congenital,  313 
metasyphilitic  disease  in,  329 
pathology  in,  314 
pseudoparalysis,  326 
spastic  paralysis  in,  333 
in  the  third  generation,  336 
of  the  nervous  system,  50 
acquired  late  in  life,  51 
difference  between  clinical  and 

anatomical  recovery,  159 
frequency   of    early   involve- 
ment, 50 

late  involvement,  50 
influence  of  treatment  on,  56 
influence  of  degree  of  primary 
and  secondary  syphilis  on 
later  developing  brain  nerv- 
ous syphilis,  58 
influence  of  other  etiological 

factors,  59-60 
special  toxicity  of,  52 
neurosis  and  psychosis  in,  166 
Syringomyelia  and  syphilis,  248 
System  disease,  in  syphilis  of  the  spinal 

cord,  218,  255 

difference  between  system  and 
pseudosystem  disease,  281 

Tabes  and  syphilis,  antispecific  treat- 
ment in,  373 

atrophy,  optic,  in,  103 

atypical  forms  of,  273 

central  gliosis  in,  249 

combination  of  tabes  and  syph- 
ilitic disease,  265 

in  congenital  lues,  264 

conjugal,  261 

isolated  gastric  crises  in,  274 

late  development  of,  263 

lightning  pains,  263 

influence  of  treatment  on  the 
development  of,  56 

migraine  in,  204 

ophthalmoplegia  interna,  133 

pseudotabes  syphilitica,  275 

pupil  anomalies  in,  274 

sterility  in,  263 

the  four  reactions  in,  352 

theories  of  origin  of,  Edinger's, 

271 

Marie's,  268 
Strumpell's,  266 
Taboparalysis,  52 

Temperature  in  specific  basilar  men- 
ingitis, 97 


Therapy  in  nervous  syphilis,  356 
bath  resorts,  367 
chronic     intermittent     treat- 
ment, 365 

of  congenital  syphilis,  375 
diagnostic  importance  of,   12 
duration  of  specific  treatment, 

364 

mercury  and  iodid,"358 
mercury   in    nonspecific    dis- 
ease, 363 

of  metasyphilitic  disease,  373 
optic  atrophy  a  contra- indica- 
tion, 366 

psychic  treatment,  368 
refractory  behavior  of  specific 
processes  to   mercury   and 
iodid,  366 

surgical  treatment,  370 
therapeutic     indications     for 
individual  forms  of  nervous 
lues,  368 
tonic,  368 
treatment    of    primary    and 

secondary  lesions,  357 
salvarsan  in,  376 

complications  following  admin- 
istration of,  380 
in  congenital  lues,  385 
dosage  of,  385 
in  paresis,  384 
in  tabes,  384 

influence  on  syphilogenetic  dis- 
ease, 382 
is  salvarsan  injurious  to  nervous 

system?  380 
intramuscular  and  subcutaneous 

injections,  376 
intravenous  method,  377 
recapitulation,  383 
Tractus    optici,    specific    disease    of, 

110 

Trauma,  influence  of,  on  the  develop- 
ment of  nervous  syphilis,  59,  357 
Triplegia  in  specific  arteritis,  68 

in  specific  cerebrospinal  meningi- 
tis, 294 

Trypanosomiasis,  relation  of,  to  pare- 
sis, 190 

Tuberculosis  of  central  nervous  system, 
similarity  between  syphilis 
and,  5,  11 

with  syphilis,  as  cause  of  nerv- 
ous disease,  17 

differential  diagnosis  from  men- 
ingitis of  the  convexity,  94 
of  the  base,  141 
cerebrospinal  syphilis, 296 
congenital  syphilis,  329 
Tumors,  pseudo-,  106 

differential  diagnosis  from  syphilitic 

basilar  meningitis,  138 
paresis,  204 
from  spinal  syphilis,  255 


INDEX 


Uraemia,  hemiplegia  in,  73 

Veins,  specific  disease  of,  37,  214 
Vertigo  in  specific  arteritis,  62 

in  specific  basilar  meningitis,  96 
Vertebrae,  pathology  in,  15 

syphilis  of,  16,  210 

tumor  of,  and  acute  myelitis,  241 
Vision,  disturbances  of,  101 

form  of,  101 

irregularities  in  field  of,  102 

variation  in,  107 


Wassermann  reaction  in  tabes,  272 

absence  of,  in  syphilitic  arteri- 
tis, 354 

in  general  paralysis,  349 
in  recent  syphilis  without  nerv- 
ous symptoms,  347 
in  spinal  fluid,  349 
not  a  specific,  338 
principle  of,  342 
significance  of,  344,  348 
technique  of,  342 

White  substance,  disease  of,  216 


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